Correction of congenital defects of the urogenital system.
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Very-long-chain acyl-coenzyme A dehydrogenase deficiency is an inborn error of fatty acid metabolism. The clinical presentation of this disease in children is either a severe form with onset of symptoms in the first months of life, cardiomyopathy, metabolic acidosis, myopathy and a high mortality, or a less severe form manifesting mainly with hypoglycaemia. Perioperative fasting and (even emotional) stress can trigger metabolic decompensation through the altered metabolism of endogenous fatty acids resulting in hypoglycaemia, acute cardiac and hepatic dysfunction and rhabdomyolysis. We report the perioperative management of a 9-year-old boy suffering from the severe form of this disease who underwent circumcision. Metabolism was kept stable in this child by using a glucose--electrolyte infusion throughout the perioperative period to avoid the biochemical consequences of fasting and a benzodiazepine--opioid technique combined with regional anaesthesia to minimize the stress response. Considering reports about a possible interference of propofol with fatty acid oxidation and to avoid the unnecessary administration of fatty acids, propofol should not be used in these patients.
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BACKGROUND/PURPOSE: The authors reviewed 60 cases of buried penis, treated in a single institution with postoperative follow-up for at least 6 months. The review was aimed at providing information that might help to optimize the results in future surgical correction of this uncommon, but not rare, congenital disorder. METHODS: From January 1, 1989 to December 31, 1998, 62 boys with buried penis were treated with 1 of the following procedures: group 1A (n = 6), preputial unfurling alone; group 1B (n = 8), modified preputial unfurling; group 1C (n = 12), penoplasty devised by the first author with preservation of the preputial skin; and group 2 (n = 36), penoplasty with trimming of the inner preputial skin. Postoperative follow-up of more than 6 months after operation was achieved in 60 of 62 patients for a total of 25 patients in group 1 (A through C) and 35 patients in group 2. RESULTS: Recurrent buried penis developed in 8 of 60 patients (13%), and redundant penile skin with or without lymphedema occurred in 18 (30%). The complications occurred in 18 of 25 patients (72%) in group 1 (A through C) but in only 8 of 35 (23%) in group 2. The difference was significant (P =.001). Most of the complications were mild and acceptable. A second procedure was required in 5 of the 25 patients in group 1 but in none of group 2. The second procedures were required to correct recurrent buried penis in 1 and to resect excess redundant penile skin in 4. CONCLUSION: The superior results achieved in group 2 suggest that in addition to penoplasty with adequate fixation of the unfurling prepuce, resection of excess inner preputial skin is required to achieve an optimal outcome in children requiring surgical correction of buried penis.
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The authors report on a course of malignant hyperthermia (MH) in an almost 5-years old boy. In the past, he had been anaesthetized two times with halothane without complications. The causative triggering agent was sevoflurane, a new user-friendly substance for paediatric anaesthesia. Forty five minutes after induction of anaesthesia he developed symptoms of a MH-crisis with increase in endexspiratory CO2 up 87 mmHg and followed by an increase in heart rate up to 160 beats/minute. The blood gas analysis showed a respiratory and metabolic acidosis. The timely administration of dantrolene rapidly reversed the life-threatening signs and prevent progression of the disease. It is apparent that monitoring of endtidal carbon dioxide by means of capnometry is of crucial importance in detecting MH at an early stage, and appropriate treatment is being instituted more promptly. By such early recognition, and treatment with dantrolene, we can reasonably except a further decrease in mortality and morbidity of this enigmatic disorder.
Paraphimosis in uncircumcised or incompletely circumcised children is a serious and painful condition requiring prompt reduction to prevent possible necrosis of the glans or urinary obstruction. Techniques described to reduce oedema distal to the constricting ring include application of ice packs, compressive elastic bandages, and making a dorsal slit which necessitates later circumcision. We have used a simple "puncture" technique to treat successfully an uncircumcised 12-year-old boy with severe paraphimosis. An 18 gauge hypodermic needle was used to puncture the oedematous foreskin at multiple sites, followed by gentle manual compression. This resulted in rapid diminution of the swelling, permitting easy manual reduction of the prepuce. Circumcision can then be performed if so desired.
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Acute urinary retention often is secondary to obstruction of the bladder or distal genitourinary system, which may be induced by any of a number of medical or surgical conditions. Treatment varies according to the cause.
OBJECTIVE: The present study, conducted in Hong Kong, aimed to evaluate the effect of topical steroid in non-retractile prepubertal foreskin by a prospective, randomized, double-blind design. MATERIAL AND METHODS: 137 boys with non-retractile foreskin were randomized to betamethasone (n = 66) or placebo (n = 71 ) for 4 weeks with application of the cream twice daily. Non-responders to treatment were offered steroid treatment for a further 4 weeks. RESULTS: The mean pretreatment grade of the foreskin in the steroid and control groups was 5.08 +/- 0.66 and 4.97 +/- 0.70, respectively. At the 4-week follow-up, 49 of the former (74%) had a retractile foreskin (grade less than or equal to 3, mean 2.38 +/- 1.41). In contrast, only 31 of the control group (44%) had a retractile foreskin (less than or equal to 3, mean 3.55 +/- 1.55) (p < 0.001). Only 14 boys were circumcised because 43 of the remaining 57 boys had a retractile foreskin after 4 weeks of treatment. CONCLUSION: when treatment is necessary, application of topical steroid as a first line of treatment may avoid surgery in almost 90% of cases.
We report a case of a 3-month-old boy with a megaprepuce presented since birth. Certain aspects of the aetiology, denomination, diagnosis and treatment are discussed. We compare our case with the three unique cases published in the literature.
Dyspareunia--painful intercourse--is a common cause of sexual difficulty. The following paper discusses, for both males and females, the common causes of dyspareunia and in many instances the appropriate treatment. Painful intercourse for the male is related either to the prepuce, glans penis, penile shaft, testicles, or urethra and prostate gland, whereas painful intercourse for the female is related to the vaginal opening, clitoris, vagina, or uterus, tubes, and/or ovaries. Each of the above is discussed.
In a randomized, double-blind study, we administered placebo and flumazenil to 40 healthy Chinese boys, aged 3-12 yr, undergoing circumcision. The children received midazolam 0.5 mg kg-1 orally for premedication and 0.5 mg kg-1 i.v. during induction. After operation the patients were given 0.1 ml kg-1 of a blinded solution followed by 0.05 ml kg-1 min-1 until either they awoke or the 10-ml ampoule of solution was empty. Efficacy of antagonism of midazolam was assessed by times to eye opening and self identification, modified Steward coma scale, a post-box toy completion-time ratio and qualitatively by an independent observer. The difference between flumazenil and placebo was both clinically and statistically different in the first 2 h. Children receiving flumazenil awoke approximately four times faster and identified themselves nearly three times sooner; 65% of this group could complete the post-box toy at 10 min, compared with none of the placebo group. There were no cases of resedation, but one child did not awaken for 30 min after i.v. administration of flumazenil 1.0 mg. The mean total dose of flumazenil administered was 0.024 (SD 0.019) mg kg-1. Flumazenil rapidly antagonized midazolam-induced hypnosis in children and was associated with minimal change in cardiorespiratory variables.
PURPOSE: The trapped penis results from cicatricial scar formation over the glans after circumcision. Management of this problem has been surgical either by incision of the cicatrix or formal surgical reconstruction. We report a series of neonates with trapped penises which were effectively managed primarily with topical steroid cream with operative intervention reserved for failed cases. MATERIALS AND METHODS: A retrospective study of 14 neonates who presented with a trapped penis treated with topical betamethasone cream was performed. Each child underwent topical application of 0.05% betamethasone 3 times daily for 3 weeks in conjunction with manual retraction that continued after the steroid course. The need for surgical intervention was assessed during and after the treatment period. RESULTS: All 14 boys were evaluated by the pediatric urologist within 4 weeks of circumcision. Each child had a trapped penis with a dense cicatrix of the residual foreskin distal to the glans. All parents were compliant with the regimen. There were no untoward effects of topical steroid application. Following therapy 11 boys (79%) demonstrated softening of the cicatrix with easy exposure of the glans in 2 and mild persistence of the cicatrix amenable to vertical relaxation incision in 2. The cicatrix persisted in 3 boys (14%) who required formal operative repair at ages 5 to 8 months. CONCLUSIONS: The combination of topical betamethasone and manual retraction is effective in managing the trapped penis. This combination may affect complete resolution of the condition in the majority of patients. Otherwise, this regimen may release the closing cicatrix and allow for simple incision of the constricting phimotic ring, thus reducing the need for formal surgical repair.
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A simple technique is described to correct preputial stenosis, based on the underlying anatomy of the lesion. The resulting prepuce is absolutely normal in appearance and function.