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Biomedical subjects

A Wakim

Publications and source records attributed to A Wakim.

13 recordsLinked to original sources

The AP diameter of the pelvis: a new criterion for continence in the exstrophy complex?

OBJECTIVE: Reconstructive surgery of bladder exstrophy remains a challenge. By using CT of the pelvis, we suggest a new pre- and post-operative investigative procedure to define the AP diameter (APD) as a predictive criterion for continence in this anomaly. PATIENTS AND METHODS: Three axial CT slices were selected in nine children with exstrophy who had undergone neonatal reconstructive surgery. The three levels selected were the first sacral plate, the mid acetabular plane and the superior pubic spine. We used combined slices to measure: APD = distance between the first sacral vertebra and the pubic symphysis. Pubic diastasis (PD). Three angles defined on the transverse plane of the first sacral vertebra--iliac wing angle, sacropubic angle and acetabular version. RESULTS: In exstrophy, the angles demonstrate opening of the iliac wings and the pubic ramus, and acetabular retroversion compared to controls. Comparisons between controls, continent and incontinent patients reveal that in continent patients, APD increases with growth and seems to be a predictive criterion for continence, independent of diastasis of the pubic symphysis. CONCLUSIONS: We believe that CT of the pelvis with measurements of the APD should be performed in all neonates with bladder exstrophy before reconstructive surgery and for better understanding of the malformation. The APD seems to be predictive and may be a major criterion for continence, independent of PD.

Bladder Exstrophy↗

Evaluation of the bony pelvis in classic bladder exstrophy by using 3D-CT: further insights.

OBJECTIVES: To provide a complete look at the bony pelvis in children with classic bladder exstrophy: dimensions, orientation, and relationships. METHODS: Three-dimensional computed tomography was used in 6 boys and 1 girl, 5 of whom underwent primary closure and 2 who underwent reclosure at 4 and 8 months. These exstrophy pelves (intrapelvic angles and osseous dimensions) were compared with 26 age and sex-matched controls. RESULTS: The iliac wing angle was 11.4 degrees larger in the classic bladder exstrophy cases. The sacroiliac joint angle was 9.9 degrees more externally rotated in the exstrophy cases. The pelvis was rotated 14.7 degrees in the superoinferior plane in the exstrophy cases. The mean pubic diastasis was 4.2 cm (0.6 cm in controls). The inter-triradiate distance in the patients with classic bladder exstrophy averaged 6.0 cm (4.2 cm in controls). CONCLUSIONS: These new findings provide a better understanding of the bony pelvis, especially its posterior portion, in patients with classic bladder exstrophy. The results of this study revealed the orientation of the sacroiliac joints to be more externally oriented than previously thought and the pelvis to be rotated inferiorly, a previously unknown observation. Both of these factors will be important in the planning of newer osteotomies and pelvic reconstruction.

Adult↗

Is there an interchromosomal effect in reciprocal translocation carriers? Sperm FISH studies.

Chromosome translocations have been known to affect disjunction of chromosomes unrelated to the translocation in the mouse and in Drosophila. However, in humans, an interchromosomal effect in chromosome translocations has not been demonstrated. The availability of techniques that allow the study of nondisjunction in sperm cells has permitted us to evaluate the possibility of an interchromosomal effect in male translocation heterozygotes. In this study, multicolor fluorescence in situ hybridization was used to determine levels of disomy for the clinically relevant chromosomes X, Y, 13, 18, and 21 in 332,858 spermatozoa from nine reciprocal translocation heterozygotes and nine controls with normal karyotypes. The specific translocations studied were as follows: t(10;12)(p26.1;p13.3), t(2;18)(p21;q11.2), t(3;19)(p25;q12), t(5;8)(q33;q13), t(11;22)(q23;q11), t(3;4)(p25;p16), t(8;9) (q24.2;q32), t(10;18)(q24.1;p11.2), and t(4;10)(q33;p12.2). Comparisons of disomy rates between carriers and controls were performed by using the Mann-Whitney test. Our results showed that the rates of sex chromosome hyperhaploidy were similar in controls (0.21%) and in translocation carriers (0.19%). Similarly, the frequencies of disomy for chromosomes 13, 18, and 21 did not differ significantly between controls and carriers (0.05% versus 0.08%, 0.07% versus 0.03%, and 0.14% versus 0.20%, respectively). Sex chromosome nondisjunction was more common than nondisjunction of chromosomes 13 and 18 both in controls (P=0.0057) and in carriers (P=0.0008). Similarly, the rates of chromosome disomy for chromosome 21 were higher than those for chromosomes 13 and 18 in both controls (P=0.0031) and translocation carriers (P=0.0057). In our study, the excess of chromosome 21 disomy versus disomy of the other autosomes was more pronounced in carriers than in controls. Thus, although the difference of disomy 21 between controls and carriers was not statistically significant, it is worthy of attention.

Adult↗

Meiotic products of two reciprocal translocations studied by multicolor fluorescence in situ hybridization.

The sperm products of two male carriers of reciprocal translocations were studied by fluorescence in situ hybridization (FISH) using a combination of three probes for each translocation. One patient carried a t(2;18)(p21;q11.2), the other a t(8;9)(q24.2;q32). The probes selected included a centromeric marker for each chromosome involved in the translocation plus a third probe distal to the translocation breakpoint of one of the translocation chromosomes. This assay identifies alternate, adjacent 1, adjacent 2, and 3:1 types of meiotic products. It allows the identification of recombination events and also estimation of the frequency of diploidy. For the t(2;18), the frequency of normal and balanced sperm and of adjacent 1, adjacent 2, and 3:1 products was 43.6%, 29. 8%, 10.5%, and 12.8%, respectively. Similar segregation patterns had been reported for this donor by direct sperm karyotyping of sperm cells. For the t(8;9), the frequency of normal and balanced sperm and of adjacent 1, adjacent 2, and 3:1 products was 44.4%, 41%, 3.1%, and 9.4%, respectively. The frequency of complementary adjacent 1 products was statistically different in both the t(2;18) (P < 0. 0001) and the t(8;9) (P < 0.0001) carrier. When the number of adjacent 2 products with one translocation chromosome (regardless of normal or derivative) was compared to the number of adjacent 2 products with the second translocation chromosome (again, regardless of normal or derivative), no statistical difference was noted for either the t(2;18) (P = 0.32) or the t(8;9) (P = 0.69). Recombination events within the interstitial segment of chromosome 2 were statistically higher than those seen in chromosome 18 (P < 0. 0001), whereas in chromosomes 8 and 9, recombination in the interstitial segments was similar (P = 0.64). The rate of diploidy was similar in both the t(2;18) (0.5%) and the t(8;9) (0.6%). Thus, FISH provides chromosome information on the sperm products produced by translocation carriers, although it cannot provide an assessment of the full chromosome complement of the spermatozoon.

Adult↗

Use of expanders for protection of viscera during irradiation of pelvic tumors in children.

The authors describe a new technique for radioprotection of pelvic viscera by placement of expanders in the subperitoneal area before irradiation of pelvic tumors. They use round shape sub-cutaneous expanders, the volume corresponding to children's weight. Seven patients were treated with excellent results, and minor complications: 2 rectitis due to unsatisfactory displacement of rectum by the balloon. There were no complications related to the device itself. The prosthesis was easy to withdraw. This method seems a safe and effective technique in protecting viscera.

Acetabulum↗

[The surgeon facing malignant non-Hodgkin's lymphoma of the abdomen in children].

From February 1981 to December 1988, 30 children with Abdominal Malignant Non Hodgkin's Lymphoma (NHL) have been followed and treated in the Department of Pediatric Surgery of Saint Vincent de Paul's Hospital. The place of surgery in the Diagnosis, Evolution and Treatment of NHL is defined. In the diagnostic Stage, Surgery should be a fortuitous event, as in some cases of acute Intestinal Intussusception, or Appendicectomy, where lymphoma is an unexpected discovery. In all other cases, a cytologic study of ascites and/or pleural effusion that should be searched, can give a quick and reliable Diagnosis. Exceptionally, a complication of chemotherapy, as a peritonitis, with or without intestinal perforation, require an intervention. Most often in cases of already diagnosed and treated NHL, an abdominal residual mass have to be removed surgically and submitted to careful pathologic examination, to determine further treatment. If in some cases, initial Diagnosis is an unexpected discovery, the treatment of NHL is essentially medical. Surgery takes place in the Remission Evaluation after conventional chemotherapy.

Abdominal Neoplasms↗

[Meconium peritonitis. The value of prenatal diagnosis].

Over an eight year period (1980-1987), fifteen cases of meconium peritonitis have been studied. As it has been reported, this pathology is still very rare. Ten pregnancies have been followed on by multiple échographies and the prenatal diagnosis of meconium peritonitis, suspected as early as 24 weeks of amenorrhea, has been established in ten cases. Among these, eight infants have been operated on before twelve hours of life, and for the ninth, surgical treatment was not advocated. The most frequent cause of the pathology is a perforation above a bowel obstruction. Two had cystic fibrosis. Of the 15 infants with meconium peritonitis, nine survived, and among these, one with cystic fibrosis and the one that has not been operated. Prenatal diagnosis in meconium peritonitis is of major interest in taking care early infants that will need an urgent surgical operation in most cases.

Diagnosis, Differential↗

[Esophageal atresia in newborn infants weighing less than 2,000 grams. Apropos of 37 case reports].

In a consecutive series of 275 infants with esophageal atresia seen at Saint-Vincent-de-Paul's Hospital (Paris) between 1971 to 1987, the authors report the experience with 37 infants weighed under 2,000 g (13.45%). 19 had severe additional malformations (51.35%) and 10 (27%) had a respiratory distress syndrome. As Abrahamson in 1972 (3), Cozzi an Wilkinson in 1975 (4), Rickham in 1981 (5) reported, according to the criteria suggested in 1962 by Waterston (1), survival rate are related to additional congenital anomalies and initial respiratory distress (pulmonary dysmaturity or pneumonia) but seems more related to maturity (small-for-date babies) than to birth weight. The authors recommend to perform a primary division of the tracheo-esophageal fistula and end-to-end esophageal anastomosis whenever possible.

Abnormalities, Multiple↗

[Congenital diaphragmatic hernia. 120 neonatal cases. Preliminary study].

120 newborns with a congenital diaphragmatic hernia (CDH) were treated during the first day after birth in the Department of Pediatric Surgery at Saint Vincent de Paul's Hospital from 1980 to 1987. The overall survival rate was 40%. The influence of prenatal diagnosis, clinical and anatomical aspects is analysed. The side and the size of the defect, the intra-thoracic liver, the intestinal malrotation and the abdominal hypoplasia are significant prognosis factors. This preliminary study suggests that the natural history of CDH may be responsible of two different diseases: a disease with an early visceral intra-thoracic growth, and a severe lung hypoplasia; another one with a secondary herniation of the intra-abdominal mass, and a better prognosis.

Atrophy↗

[Tracheomalacia in esophageal atresia].

On the basis of a series comprising 154 cases of Type III oesophageal atresia and 19 cases of Type I oesophageal atresia the authors have tried to define the place of tracheomalacia in association with oesophageal atresia. We have studied a certain number of technical and anatomical factors as well as the relation of tracheomalacia to the other complications, in order to class more precisely the therapeutic solutions available.

Cartilage Diseases↗

Osteotomy and distraction of the anterior segment of the pelvic ring in epispadias repair: case report.

We report a new therapeutic approach for bladder exstrophy and epispadias in one case of failed epispadias repair. The width of the pelvis was measured by what we defined as the anteroposterior diameter (APD) on combined transverse computed tomography (CT) scan cuts of the pelvis. The APD was half the normal value in an incontinent patient with failed epispadias repair. He underwent a supraacetabular osteotomy of the pelvis with progressive anterior distraction of the anterior segment of the pelvic ring. Four months later, hardware was removed, and the APD was near normal value. Within 9 months of follow-up, the patient was dry day and night. We believe that in patients with failed exstrophy and epispadias repair, APD seems to be a predictive criterion for continence, and results of the reconstructive surgery with osteotomy should be improved by distraction of the anterior segment of the pelvic ring.

Acetabulum↗

The pelvis of fetuses in the exstrophy complex.

By using a three-dimensional computed tomography (CT) scanner, we compared the anatomic features of the pelvis of three fetuses of same gestational age, one with a normal pelvis representing the reference model, one with classic bladder exstrophy, and one with cloacal exstrophy. The tomography slices were selected at the same levels for each case. Three angles expressing external opening of the pelvis were defined. Comparing normal and abnormal pelvises allowed definition of three criteria for the correction of the malformation: (a) the sum of the differential angles gives the amplitude of the correction needed; (b) a supraacetabular osteotomy appears to allow best closure of the pelvic ring; (c) only three slices of a CT scan are needed, which cannot be harmful, especially for neonates. Therefore, we believe that a CT scan of the pelvis should be performed whenever an osteotomy is planned in the surgical reconstruction of bladder and cloacal exstrophy.

Anthropometry↗