The authors relate their experience with 8 cases of management of post-traumatic cubitus varus in the Africa child. In this series the mean varus angle, sense stricto, irrespective of the physiological valgus, was 24 degrees with extreme values ranging 10 degrees-45 degrees. Indication for surgery has always rested with the true varus value and with the degree of resulting esthetic and functional prejudice. Resection of an external bone wedge combined with synthesis using two crossed rods yielded good results, and rehabilitation was started as early as the first month after surgery.
Fifty-six pediatric patients (2.5 months-13 years old) presenting with unilateral inguinal hernia underwent intraoperative diagnostic pneumoperitoneum (Goldstein test) to evaluate the controlateral groin. Fourteen patients (25%) had positive results and underwent controlateral hernia repair. The positivity is found more frequent in females (37.5%) and in the left sided hernias (50%). The percentage of positive tests has found to be inversely proportional to the age of the patient which is 33% for children under 6 months of age and 18% over 24 months. It is also observed that none of the children with negative test admitted with the complaint of hernia within two years following the operation. Intraoperative diagnostic pneumoperitoneum is a rapid, safe and accurate means to evaluate the contralateral groin in children presenting with a unilateral inguinal hernia. The test eliminates the high negative exploration rate and possible injury to cord structures with routine bilateral exploration.
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)
Two cases of prenatal detection of congenital biliary cysts are reported. Prenatal and postnatal ultrasonography demonstrate a solitary sonolucent cystic lesion. The differential diagnosis in fetus and infant includes any abdominal cystic lesions: ovarian cyst, duplication cyst, cystic lymphangioma, choledocal cyst and other congenital cysts of the liver. In opposite to the difficulty of diagnosis, the choice for treatment is easy: surgery is the only way to allow histologic diagnosis and to avoid any relapse or complications.
The authors report two rare cases of pygomelus monster. The appearance and operative procedures undertaken are described. Pygomelus and caudal duplication are often mixed in literature. This monstruosity is classified in the twin monsters, but minor forms any be discussed with saccro-coccygeal teratomas and reduplication of lower limbs.
Seventeen sacrococcygeal teratomas with prenatal diagnosis are studied. The diagnosis was made at 27 weeks. Three fetuses died in utero, two of them from the evolution of the tumor. Two died post-natally from hemorrhage and one past natally because of his prematurity. From the eleven long term survivors, one developped a recurrence and five sequelae. This study confirms the poor prognosis of fetal sacrococcygeal teratomas. The 2 major prognosis factors are the growth of the tumor and the occurrence of fetal congestive heart failure. That congestive heart failure is diagnosed by Doppler echocardiography before hydrops and placentomegaly who are of very poor prognosis. The extraction of the fetus is mandatory if these features appeared after 30 weeks. Before this term, fetal surgery is the only effective treatment. At a long term, the risk of recurrence is low but implies a long term surveillance of the level of alfa foeto protein.
Four cases of Carpal Tunnel syndrom during childhood are reported. Two of them are post traumatic cases; for one child, the syndrom was caused by hypertrophic cal six months after trauma. The other child has been operated because of a median and ulnar nerve deficit syndrom, which appeared one month after fracture.In the third observation, the authors have been an anormal muscle just in front of annular anterior ligament of the carpus during surgery. This muscle was assimilated to hypertrophic Palmaris Brevis muscle. In the last observation, the adolescent had a Poland syndrom with Sprengel malformation.In this paper, the authors point out clinical particularities due to musculo-tendinous malformation which can lead to Carpal Tunnel syndrom.
True hermaphroditism is an usual cause of ambiguous genitalia. In some social areas, the diagnosis is often late and rised as pubertair abnormaly. We report a case of a 12 years old Sicilian child, seen in 1975, raised as a boy and whose the main comptain was a gynaecomastia. Clinical and paraclinical investigations revealed a small testicle on the one side and on ovary with an uterus and an obturated tube ont the opposite side. A small recurvated penis, partially adherent to the scrotum is noticed. A structure embryologycally close to a vagina is also found behind the bladder. Cytogenetic structures showed a mixte karyotype: mosaicim 46 XX/46 XY with a ratio of a 80/20. Hormonal assessment showed a normal level of estrogene while testosterone is below the inferior threshold. A surgical treatment is carried out in three steps: removal of the internal female organs and testicular prosthesis replacement in the one side after castration, reconstruction of the recurvated penis and replacement of the other testicular prosthesis, and finally construction of the anterior urethra. Since the very first step of the surgical management and adjuvant hormonotherapy (testosterone) is administrated in order to decrease the gynaecomastia but also to allow the norma growth of the male organs. We discuss the benefice of a such therapeutic option in the true hermaphroditism lately diagnosed regording to organic and psychological data. We also point out the difficulty in therapeutic choise, mainly when the patient has raised as a boy. The follow-up in this case in 15 years.
Report of the observation of a female newborn presenting a complex malformative set: - distal anorectal malformation without fistula; - bladder exstrophy without epispadias, with cutaneous bridge; - congenital urethrovaginal fistula; - malformatives sexual ambiguity with bulky genital tubercle, whole urethra, urethral meatus on the apex of the genital tubercle, associated with a mid labial genital joint and a hight genital orifice; - unilateral multicistic kidney; - vertebral abnormalities with supernumerary thoracolumbar vertebrae; - no caryotype abnormalities and no familial pathological history; - no endocrine abnormalities. The therapeutic multistage program permited a good reconstruction of the different lesion, particularly with bladder functioning which in hope a good continence with two years of follow-up. No similar case is reviewed in the literature.
