1/33. Persistent anaphylactic reaction after induction with thiopentone and cisatracurium. A 6-year-old boy presented for surgery for phimosis. The anaesthetic technique included intravenous induction with thiopentone and neuromuscular blockade with cisatracurium. Severe persistent bronchospasm and central cyanosis followed the administration of these drugs. A continuous i.v. infusion of epinephrine at 0.2 microg. kg(-1) x min(-1) was necessary to break the severe refractory bronchial hyperresponsiveness. There was no previous exposure to anaesthetic drugs and no definite family history of allergy. Through increased serum eosinophil cationic protein, tryptase and histamine levels and IgE levels specific to cisatracurium, we demonstrated an IgE-mediated anaphylactic reaction to cisatracurium in the child's first exposure to this new neuromuscular blocking agent. Anaphylactic reactions to new anaesthetic drugs may be challenging to recognize and treat during general anaesthesia in children. The pathogenesis, diagnosis and management of life threatening persistent allergic reactions to intravenous anaesthetics are discussed. ( info) |
2/33. Primary lymphedema of the penis: surgical correction by preputial unfurling. Primary lymphedema of the penis (PLP) is a rare disease. We report a case in a 2-year-old, uncircumcised boy where the uninvolved inner preputial skin was unfurled to cover the penile shaft. The uninvolved inner preputial skin is often elongated, and provides a suture-free cover of sufficient length for the small penile shaft of pediatric patients. ( info) |
3/33. Preputial calculi: a case report. The patient was a 92-year-old male whose chief complaint was urinary retention. The x-ray film showed multiple overlapping calcification shadows in the penile region. renal insufficiency was speculated to be due to post-renal obstruction. Under the diagnoses of closure of the preputial orifice by balanoposthitis followed by urinary retention and preputial calculi, an urgent dorsal incision of the prepuce was made. Then, stone removal and indwelling catheter placement were performed. Renal function recovered soon after the operation, and the patient could urinate freely without catheterization. This case reminds us of the significance of surgical treatment for phimosis in elderly patients. ( info) |
4/33. Perioperative management of a child with very-long-chain acyl-coenzyme a dehydrogenase deficiency. 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. ( info) |
5/33. Cicatricial pemphigoid-induced phimosis. Cicatricial pemphigoid is an autoimmune bullous disease that has a predilection for mucous membranes and often results in scarring. We describe a case of cicatricial pemphigoid with severe involvement of the glans penis, which, despite systemic therapy, led to phimosis. ( info) |
| We report a 5-week-old boy who developed severe hyponatremia and hyperkalemia secondary to acute pyelonephritis. The patient presented with non-specific signs, including poor appetite, failure to thrive, and dehydration. An endocrinological evaluation led to a diagnosis of pseudohypoaldosteronism. The patient had phimosis, but no congenital urinary tract malformations. Outflow obstruction secondary to the phimosis appears to have caused pyelonephritis, and renal inflammation decreased responsiveness to aldosterone transiently. ( info) |
7/33. phimosis as a cause of the prune belly syndrome: comparison to a more common pattern of proximal penile urethra obstruction. The pathogenesis of the prune belly syndrome (PBS) remains controversial, but two theories predominate. The first theory supports an obstructive phenomenon early in gestation leading to irreversible damage to the genitourinary tract and abdominal wall. The second theory suggests mesodermal injury between the 6th and 10th weeks of gestation as the primary abnormality. This paper reports of two fetuses with the PBS phenotype that were examined postmortem at our institution. Thorough examination of the lower urinary tract allowed demonstration of anatomic obstruction of the urethra in both cases. One case illustrated a relatively common pattern of proximal penile urethral obstruction, a flap-like obstruction between the prostatic and penile urethra. The other case provided what we believe to be the first description of PBS caused by severe phimosis. ( info) |
8/33. The value of serological tests for syphilis in atypical genital ulcer disease. A 45-year-old heterosexual man presented with phimosis three weeks after arriving in this country from india. Examination under anaesthesia and circumcision revealed a large ulcer on the ventral aspect of the penis around the fraenum. Histopathological examination of a biopsy taken from the ulcer revealed granulation tissue with inflammatory cell infiltrate. Specific and non-specific serological tests for syphilis carried out four weeks after the initial presentation showed high titres. The importance of serological tests for syphilis in genital ulcer disease, especially in atypical presentations, is discussed. ( info) |
9/33. Kindler syndrome. Kindler syndrome is a rare genodermatosis characterized by acral bullae and photosensitivity. The photosensitivity improves with advancing age and results in progressive poikiloderma and cutaneous atrophy, and many additional features have also been described. This report describes two male Kindler syndrome patients with classical features of acral blistering and photosensitivity in childhood, and subsequent development of poikiloderma, leukokeratosis of oro-ano-genital mucosae, phimosis and meatal stenosis. The first patient had additional ophthalmic features of chronic simple conjunctivitis caused by persistent irritation, multiple stromal nebular corneal opacities and thickened corneal nerves. The second patient showed skeletal changes, namely a dome-shaped skull (turri-cephaly), bifid fourth rib, missing fifth rib, short fourth and fifth metacarpals and mandibular abnormalities. This is the first report of such ophthalmic and skeletal features of Kindler syndrome. ( info) |
| We report on a 47-year-old man with oral and genital lichen planus. After some months of the disease, increasing phimosis developed which had not been present before. Retraction of the foreskin was now impossible and sexual intercourse was painful. Treatment with triamcinoloneacetonide and etretinate ameliorated the phimosis but the patient was still not comfortable and circumcision was performed. histology from the foreskin revealed the typical picture of lichen planus. No features of lichen sclerosus et atrophicus were present. This is the first published observation of phimosis as a result of lichen planus. ( info) |