Chirurgie pediatrique最新文献

Hereditary angioneurotic oedema is an autosomal dominant disorder associated with serum deficiency of functionally active C1 inhibitor protein (type 1) or normal serum level of functionally deficient C1-inhibitor (type 2). These biochemical abnormalities induce a complement activation which leads to episodic swelling of interstitial tissues usually of the abdominal viscera and of the upper airway with resultant asphyxia. Vasoactive peptides from the degradation of component C2 of the complement along with an activation of basophils by chemotactic substances from the degradation of C3 and C5 are the main mechanisms involved in vasodilatation and swelling. Attacks of angioneurotic oedema, sometimes fatal when involving the upper airway, can occur during any but usually during ear-throat-nose, dental or facial surgery. This article describes the pathophysiology and the main features of the disease in children. It reviews the different treatments used to avoid attacks or to cure an attack of angio-oedema once it has begun, both during planed surgery and during emergency surgery.

A series of 161 boys with 178 impalpable testes is reviewed. Extended inguinal exploration was the main form of investigation and treatment. Hormone injections and studies were performed in bilateral cases. Sonography was unhelpful. Laparoscopy was not used. Impalpables testes account for 7% of all boys with an anomaly of testicular descent and 8.5% of operated boys. One hundred and twenty six testes were found, 58 in the abdomen, 68 in the inguinal canal. Thirty one nodular vestige were found and all 25 with histopathology were spermatic duct residue. In 21 boys no trace of testes or spermatic duct remnant were found. Ninety five testes were successfully brought to the scrotum with one (85) or two (10) stage procedure. Anomalies of the upper urinary tract in 13 of 21 cases without testes or spermatic duct remnant were very rare. In bilateral cases it looks better to search a testes whatever the biological responses to gonadotropin stimulation.

Intervertebral space calcification (ISC) is infrequent, it involves different levels of the spine. Anterior prolapse of the calcification at cervical level may get dysphagia, exceptional and transitory sign of the disease. "Crisis" of pain is a criteria of removing of calcification existing long time before its detection.

The authors report the case of an 3 months old caucasian boy which had an extrahepatic biliary atresia associate with a congenital biliary cyst and an anomalous pancreaticobiliary junction. The patency between gallbladder and duodenum permits the operative opacification of this long common pancreaticobiliary channel. The congenital biliary cyst was located on the upper part of the common bile duct and was obstructed by biliary concretions. Unfortunately, no patency between intrahepatic bile ducts and this cyst was found. Because this long common pancreaticobiliary channel may cause biliary dilatation or biliary cancer, we performed an hepatic portoenterostomy rather an hepatic portocholecystostomy. A few weeks later, the bile flow was restored and the jaundice decreased. With a follow up of 18 months, this boy is currently anicteric, despite the hepatic fibrosis found on the operative biopsy. The association extrahepatic biliary atresia-long common pancreaticobiliary channel is uncommon and only 20 cases were reported. In our case, the pancreatic juice reflux into the biliary tract might have caused the dilatation of the common bile duct and the biliary atresia by chronic obstructive cholangiopathy.

Mucosal prolapse is a frequent sequela after surgical treatment of the high type of anorectal malformation whatever was the technic used for the pull-through operation. The authors report six cases treated with the spiral flap described by Millard. The initial procedures had been a Romualdi-Rehbein pull through in three cases with secondary revision of anoplasty and a Stephan's operation in the three other cases. Before Millard's plasty, all the patients were continent and two of them had an associated stricture. The anatomical result was good in all the cases with reconstruction of a cutaneous anal funnel and no stricture. Improvement of continence was noted in two cases, uncertain in two. Incontinence remained unchanged in two other cases. The advantages of the Millard's plasty are analysed. The prevention of mucosal prolapse is possible in the initial pull through operation using the same plasty or whatever plasty which provides a cutaneous anal canal.

Inherited adenine phosphoribosyltransferase (APRT) has a recessive transmission. When it is very important, adenine can't be restored into nucleic acids pool and will changed into 2,8-dihydroxyadenine (2,8-DHA) by xanthine oxidase. To date in all countries but Japan, 2,8-DHA urolithiasis is observed only into homozygotic subjects with complete APRT deficiency Commonly, its onset is observed in childhood often dramatically. The authors report two new pediatric cases into new french families. First a 8 years old boy with spontaneous elimination of two lithiasis after right lumbar pain. Secondly an infant (nineteen months) who has presented an acute renal failure with anuria. Bilateral lithiasis included into pyelourectal junctions have been pulled out by bilateral surgical pyelotomy. In each case, lithiasis were radiolucent and diagnosis made by ultrasonography. The uric acid metabolism was normal and it is the infra red spectrophotometric study of stones that had recognised the 2,8-DHA component. In the second case, bilateral residual lithiasis have been broken by piezoelectric extra-corporeal lithotripsy with good tolerance and favorable result. The two children received preventive treatment. After 36 and 19 months they have no recurrence. In the literature, the frequency of 2,8-DHA lithiasis is very more low than the theoretical of homozygotics in population (1/100,000). The common confusion with uric lithiasis is one possible explanation. So spectrophotometric study of radiolucent stones was meant to be realised when uric metabolism is not disturbed.(ABSTRACT TRUNCATED AT 250 WORDS)

Ano-rectal myectomy, described by B. Duhamel in 1965, is the logical therapy of rectoanal achalasia; an experience of 117 cases is reported. Clinical examination and radiological investigations have been made prior to surgery. This surgical procedure is simple, without complication. Three kinds of rectoanal achalasia are described: achalasia with structural change of the internal sphincter (fibrosis, sclerosis); achalasia with a functional sphincteric disorder (with normal histology); achalasia with intrinsic innervation abnormality. Excellent results have been observed in the two first groups but more variable successful results in the third group which concerns short segments of Hirschsprung's disease.

The usual surgical treatment of primary obstructive megaureter consists of ureteral tailoring, according to H. Hendren's procedure, and reimplantation using Politano-Leadbetter technique. The Authors report their experience with trans-trigonal reimplantation (Cohen's ureteroneocystostomy) in case of primary obstructive megaureter. This procedure has been performed either in case of tailored megaureters than in those in which ureteral tailoring was not necessary. Considering its easy handling and safety, Cohen's technique has been indicated as method of choice for ureteral reimplantation even in case of primary obstructive megaureter, basing on successful experience on the treatment of vesicoureteral reflux. A current objection to the trans-trigonal technique for obstructive megaureter is the possibility of a postoperative reflux in the reimplanted megaureter (tailored or not), because of the difficulty in obtaining an adequate submucosal tunnel (unbalanced length/width ratio). The Authors report a 3 year experience on a common surgical treatment of primary obstructive megaureter. 56 cases were treated (bilateral megaureter in 3 cases) by trans-trigonal reimplantation (Cohen's ureteroneocystostomy) in addition to ureteral tailoring (Hendren's technique) in 46 cases or Kalicinski plication in 6. Finally, in 4 cases no tailoring procedure was needed (simple excision of the restricted terminal ureter). No postoperative complications nor cystographic evidence of postoperative reflux have been recorded in all cases (follow-up = 6-36 months), remarking this technique as safe and effective.

Serpentine foot or Z-shaped foot, definite as varus of forefoot with valgus of heel, is a deformity which is advisable to separate from metatarsus varus in children. We have studied 55 serpentine feet of 31 children aged between 6 months and 13 1/2 years, observed in 20 years. We distinguished four grades of deformities. Treatment was orthopedic in 15 times, surgical in 29, with 50% of bad result, 11 feet which haven't had any treatment were in use to indicate evolution of the adductus. However, valgus of heel is transitory and secondary to forefoot rigidity, it always regressed but growth disturbance of tarsal bone occurred making lateral deviation. We insist on early radiographic diagnosis and treatment which is orthopaedic before first year of age, then surgical when first failed or in old children. It get release of metatarsal stiffness before 6 years of age, then in older we make osteotomy of 1 degree cuneiform and cuboid to correct bone deformity. Correction of hind foot valgus, realized in 24% of the surgical treatment, must be prohibited, it always made transverse tarsal instability.

The authors report a case of a twelve old young man, operated on for a ganglion cyst of the common peroneal nerve. The lesion is rare, and is characterized by an accumulation of a mucoïd substance in the sheath, compressing progressively the fibres of the nerve. The case was revealed with pain, paralysis and tumefaction of the nerve. The pathogenic is uncertain. An early treatment, consisting of evacuation of the cyst and interfascicular neurolysis through the dissecting microscope, obtains usually a good functional result.