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Biomedical subjects

M Cendron

Publications and source records attributed to M Cendron.

At least 37 records · Page 2Linked to original sources

Prenatal ultrasound characterization of the suprarenal mass: distinction between neuroblastoma and subdiaphragmatic extralobar pulmonary sequestration.

With the increased routine use of prenatal ultrasonography, subdiaphragmatic masses in the fetus are identified more frequently. Suprarenal masses often are presumed to be neuroblastoma and are removed surgically postnatally. We sought to better understand the natural history of subdiaphragmatic extralobar pulmonary sequestration, and to determine if subdiaphragmatic extralobar pulmonary sequestration can be distinguished preoperatively from neuroblastoma. The literature was reviewed for cases of prenatally diagnosed suprarenal masses that proved ultimately to be either subdiaphragmatic extralobar pulmonary sequestration or neuroblastoma. The distinguishing features of the two lesions were identified and an algorithm was created on the basis of these distinctions. Prenatally diagnosed subdiaphragmatic extralobar pulmonary sequestration is no longer rare, with one case being reported for every 2.5 cases of neuroblastoma. On prenatal ultrasonography subdiaphragmatic extralobar pulmonary sequestration usually is echogenic, is left-sided, and can be identified in the second trimester. Neuroblastoma is most often cystic, right-sided, and identified in the third trimester. In summary, subdiaphragmatic extralobar pulmonary sequestration must be considered in the differential diagnosis of the suprarenal mass identified on prenatal ultrasonography. Using the algorithm which we propose, the correct diagnosis can be determined prenatally in 95% of patients.

Adolescent↗

Bladder exstrophy: evaluation of factors leading to continence with spontaneous voiding after staged reconstruction.

PURPOSE: We performed a long-term retrospective review of patients with bladder exstrophy to evaluate the results of staged surgical reconstruction in regard to urinary continence, spontaneous voiding and preservation of the upper urinary tract. MATERIALS AND METHODS: We reviewed the charts of 42 boys and 15 girls treated at Hôpital St. Joseph for bladder exstrophy between 1965 and 1995. All patients underwent staged repair, including bladder augmentation in 7 (12%) and secondary urinary diversion in 13 (23%). Criteria for good outcome in terms of continence are defined and factors influencing outcomes are reviewed. RESULTS: A total of 38 patients (67%) achieved good or acceptable urinary continence (22 or 39% and 16 or 28%, respectively). Outcome was poor in 19 patients, including 13 (23%) who underwent secondary urinary diversion. Bladder stones, which developed in 13 patients (23%), were the most common long-term complication of bladder exstrophy reconstruction. Ten patients who underwent pelvic osteotomies ultimately had better continence and 9 are dry. Bladder neck reconstruction was performed at a later age overall (mean 10 years). Repeat bladder neck reconstruction was generally associated with poor results. The technique of bladder neck reconstruction did not appear to influence outcome. CONCLUSIONS: A carefully planned surgical reconstruction for bladder exstrophy can lead to satisfactory long-term urinary continence in most patients. Factors contributing to successful results include early bladder closure, pelvic osteotomy, adequate bladder neck reconstruction with bladder neck suspension in girls, and a motivated child and family. Alternatives to surgical reconstruction should be discussed. Ultimate predictors of outcome in bladder exstrophy repair are difficult to ascertain.

Adolescent↗

The vanishing fetal ureterocele: a cause for concern?

The natural history of fetal ureteroceles is poorly understood. We report two cases of fetal ureterocele which add to our understanding of its natural history. In both cases, the fetal ureterocele 'resolved' later in gestation. The postnatal findings emphasize the importance of an aggressive newborn evaluation despite apparent resolution of the ureterocele on prenatal ultrasound examination.

Female↗

Prenatal diagnosis and the pediatric surgeon: the impact of prenatal consultation on perinatal management.

PURPOSE: Pediatric surgeons are increasingly called on by obstetrical colleagues to counsel parents about the implications of a prenatal ultrasound finding. Our understanding of the natural history of many prenatally diagnosed surgical conditions has grown significantly in recent years. Whether prenatal surgical consultation can influence perinatal course had not been investigated. METHODS: During an 21-month period, 12,865 prenatal ultrasound studies were performed on a total of 4,551 patients, and 221 prenatal surgical consultations were obtained through a newly established fetal treatment program at a tertiary care prenatal diagnostic center. To evaluate the impact of prenatal pediatric surgical consultation on perinatal course, the authors reviewed changes in management including termination of pregnancy, in utero intervention, and altered site, mode, or timing of delivery. RESULTS: Two hundred twenty-one fetuses were referred for consultation; their 234 congenital anomalies included genitourinary (36%), thoracic (16%), intraabdominal (14.5%), abdominal wall (10.6%), neurological (9%), skeletal (6%), and head and neck (2.5%) defects; 2.5% had tumors and 2.5% were twin pregnancies. Pregnancy was terminated in 9.5% of cases, because of patient request, chromosomal abnormality, or dismal prognosis. In 3.6%, the decision to terminate was changed as a result of consultation. Site of delivery was changed as a result of consultation in 37% to facilitate postnatal evaluation and initiate immediate treatment. Mode of delivery was changed in 6.8% to prevent dystocia, hemorrhage into a tumor, as in sacrococcygeal teratoma, or to provide an emergency airway, as in cervical teratoma. The timing of delivery was changed in 4.5% to avoid further damage to fetal organs in cases of obstructive uropathy, gastroschisis, sacrococcygeal teratoma with high-output failure, and hydrocephalus. Five percent (11) underwent treatment in utero for fetal hydrothorax, obstructive uropathy, twin-twin transfusion syndrome, or lymphangioma. The overall perinatal mortality rate was 2.5%. CONCLUSION: Prenatal pediatric surgical consultation may have a significant impact on the perinatal management of the fetus with a surgically correctable congenital anomaly. Providing obstetric colleagues and families with valuable insight into the surgical management of anomalies allows fetal intervention when appropriate, and delivery in an appropriate setting, by the safest mode of delivery, and at the gestational age appropriate to minimize effects of the anomaly.

Abortion, Therapeutic↗

Renal cell carcinoma in association with tuberous sclerosis in children.

Tuberous sclerosis, a genetically transmitted multisystem neurocutaneous disorder, is associated with renal lesions in 50% of cases. The expected lesions are angiomyolipomas or renal cysts; renal cell carcinoma has been encountered in adults who have tuberous sclerosis, but is very rare in children. The authors report the case of a 5-year-old girl with tuberous sclerosis for whom atypical computed tomography findings led to the diagnosis of renal carcinoma at an early age. This experience suggests that children with tuberous sclerosis may need earlier screening.

Adult↗

Minimal hydronephrosis in the fetus: clinical significance and implications for management.

PURPOSE: We further define the natural history and management of minimal fetal hydronephrosis. MATERIALS AND METHODS: Experience with minimal fetal hydronephrosis (renal pelvic diameter 4 to 10 mm. before 20 weeks of gestation and 5 to 10 mm. from 20 to 24 weeks) was reviewed for 18 months at New England Medical Center. RESULTS: The incidence of minimal fetal hydronephrosis was 2.2% in the 5,900 fetuses screened by ultrasound. In 63% of cases prenatal ultrasound was done more than once, and revealed that dilatation of the urinary tract was stable during gestation in 31 (25%), and became normal in 35 (29%) and worse in 11 (9%). A total of 63 patients (103 renal units) followed for up to 1 year underwent postnatal sonography. Those with abnormalities were screened with a voiding cystourethrogram and renal scan, including 40 with normal postnatal ultrasound and no changes on subsequent evaluation. Of the 23 patients with abnormal findings 4 had functionally significant ureteropelvic junction obstruction and 1 had severe vesicoureteral reflux. A retrospective analysis of the sonograms of patients with minimal fetal hydronephrosis and persistence or progression of hydronephrosis revealed calicectasis, progression of minimal fetal hydronephrosis in utero and/or abnormal renal echogenicity in each fetus. CONCLUSIONS: Our study shows that in a small but significant number of fetuses minimal fetal hydronephrosis will progress, whereas in most it will resolve.

Disease Progression↗

The biological behavior of autologous collagen injected into the rabbit bladder.

Growing interest in the endoscopic treatment of vesicoureteral reflux and urinary incontinence has led to the development of safe reliable techniques that use an injectable bulking agent. A variety of injectable materials has been studied, such as polytetrafluoroethylene, fat and bovine collagen. We evaluated autologous collagen extracted from the dermis of skin for potential use in the urinary tract. Therefore, an animal model was developed to study the biological behavior of autologous collagen within the bladder. Collagen was extracted from the skin of rabbits and radiolabeled with 125iodine. An aliquot of radiolabeled autologous collagen was injected submucosally in the bladder. The animals were monitored weekly under a gamma camera. The decay of the radiolabeled collagen was recorded during 50 days. The animals were then sacrificed and the bladder wall at the site of injection was studied histologically and on electron microscopy. Decay of the injected collagen within the urinary tract was similar to control material, indicative of good stability within the bladder. A mild inflammatory response with fibroblastic and vascular invasion of the collagen implant was observed. These preliminary data suggest that autologous collagen injected endoscopically can provide a safe reliable bulking material that could be used to treat urinary incontinence and lower grades of vesicoureteral reflux in children.

Animals↗

Molecular characterization of the androgen receptor gene in boys with hypospadias.

Development of male external genitalia is dependent on androgens, and karyotypic males lacking appropriate levels of androgens or functionally normal receptors may show abnormal virilization. Mutations in the androgen receptor gene cause abnormal receptor function and diverse mutations may be associated with heterogeneous clinical signs of androgen insensitivity. In this study, we have searched for the existence of androgen receptor gene mutations carried by some patients with hypospadias. Genomic DNA samples from peripheral blood leucocytes from 21 patients with different degrees of hypospadias were studied. Analysis of the androgen receptor gene was performed by exon-specific amplification using polymerase chain reaction, single strand conformation polymorphism analysis, and direct genomic sequencing. Although a silent polymorphism was identified in exon 1 of the androgen receptor gene, the majority of patients studied (20/21) did not carry androgen receptor gene mutations. One patient with severe hypospadias and bilateral cryptorchidism was found to carry a point mutation in exon 8. We conclude that mutations in the androgen receptor gene may be carried by subset of patients with genital ambiguity presenting primarily with hypospadias, but this is not the underlying cause in the majority of cases. Characterization of this genetic defect may be important for classification and subsequent conservative therapeutic approaches for these patients.

Base Sequence↗

A fetal lamb model of partial urethral obstruction: experimental protocol and results.

The functional effects of bladder outlet obstruction in the developing urinary tract are well recognized in patients born with posterior urethral valves, in whom a spectrum of bladder dysfunction has been described. To better understand the changes occurring in the partially obstructed developing lower urinary tract, a fetal lamb model of partial urethral obstruction was developed. Fetal lambs at 90 days' gestation underwent surgical placement of a silver ring (ex utero) at the level of the proximal bladder neck, with concomitant ligation of the urachus. Control animals underwent urethral ligation only. The lambs were then allowed to go through normal gestation, and ewes were delivered spontaneously. The animals were studied between 2 and 7 days after birth. The postmortem examination showed that the ring was just distal to the bladder neck, around the proximal urethra. This resulted in gradual, partial occlusion of the urethra. Bladder weights, bladder wall thickness, and bladder capacity were significantly increased in the partially obstructed animals as compared with the controls. There was little or no upper tract dilatation in the obstructed group. This animal model, the first to produce gradual outflow obstruction in the fetus, provides a reproducible model of partial urethral obstruction. The model can be used to assay the biochemical and physiological changes found in the developing urinary tract of fetal lambs submitted to intravesical obstruction.

Animals↗

Urological aspects of intra-abdominal desmoplastic small round cell tumor of childhood: a preliminary report.

To our knowledge intra-abdominal desmoplastic round cell tumor has not previously been described in the pediatric urological literature. This lesion has only recently been recognized as a clinicopathological entity with predilection for adolescent boys. We report on 2 patients who presented with urological symptoms and a large abdominal or pelvic tumor that was biopsied at exploratory laparotomy. Complete surgical excision was impossible in both patients, who subsequently underwent multiagent chemotherapy. Histological and immunohistochemical staining are distinctive in this condition. The tumor is associated with a poor prognosis, despite multidisciplinary, multimodal therapy.

Abdominal Neoplasms↗

The desmoplastic round cell tumor: a new solid tumor of childhood.

Three patients with a new, pathologically distinct solid tumor of childhood have been treated recently. The disease is characterized by male predominance, adolescent onset, an extensive abdominal primary tumor, and aggressive metastases to regional lymph nodes, liver, and lung. Two patients presented with vague abdominal pain and the third with testicular pain. All three noted fatigue and malaise of less than two months' duration with minimal associated weight loss. Computed tomography (CT) scans of the abdomen and chest were obtained for initial preoperative staging, and then all three underwent surgical exploration. Widespread disease was found in each case. In no instance was complete tumor extirpation possible because of extensive peritoneal spread and lymphatic and hepatic metastases. Histologically, all three tumors consisted of round blue cells with a dense desmoplastic reaction and focal rhabdoid features. Immunohistochemical markers for epithelial, neural, and muscle elements were positive. Aggressive multidrug chemotherapeutic regimens were used in each case, and all three patients are alive and well but with known residual disease. We conclude that in cases of the desmoplastic round cell tumor of childhood, CT scans underestimate the extent of disease, and exploratory laparotomy is necessary for diagnosis and appropriate staging. Surgery is usually palliative because of extensive spread. Awareness of this newly recognized aggressive solid tumor of childhood is essential to define its natural history and guide the development of effective multidisciplinary therapeutic regimens.

Adolescent↗

Developmental alterations in the fetal lamb bladder subjected to partial urethral obstruction in utero.

To identify the structural changes seen in the developing bladder subjected to outflow obstruction, a fetal lamb model was developed. With this model, which attempts to reproduce conditions such as those found in posterior urethral valves, quantitative biochemical parameters were used to evaluate the effects of partial outflow obstruction on detrusor cellularity and innervation in the developing bladder. Partial urethral obstruction was created in 9 fetal sheep (90 to 100 days gestation), 6 sham operated animals serving as controls. Fetuses were delivered at term and sacrificed. Bladders were removed and weighed. Mucosa free detrusor was assayed for DNA and choline acetyltransferase (ChAT) activity, indices of cell number and cholinergic innervation, respectively. In similar specimens of detrusor, cell ploidy and nuclear size were determined by flow cytometry and nuclear morphometry, respectively. The results showed that partial urethral obstruction in utero resulted in a significant increase in bladder weight (p < 0.05) and total detrusor DNA content (p < 0.01). With the increase in detrusor DNA content, there was a parallel increase in total detrusor ChAT activity. These results suggest that early development of the bladder is altered both on a cellular level (increase in cellularity) and a neural level (increase in cholinergic nerve density) under conditions of mechanical distension.

Animals↗

Anatomical, morphological and volumetric analysis: a review of 759 cases of testicular maldescent.

Undescended testis is the most frequent disorder of male sexual differentiation affecting 0.8% of all boys by age 1 year. Anatomical and descriptive features of undescended testis lack standardization, and often do not reflect intraoperative findings. We review a large cohort of patients treated for undescended testis and analyze anatomical, morphological and volumetric findings at orchiopexy. Of 759 patients 1 month to 9 years old entered into the study between 1985 and 1990 at our hospital 55% underwent surgery between ages 8 months and 4 years, 15.5% had bilateral undescended testis and 27.6% had a nonpalpable gonad. Preoperative physical findings correlated poorly with intraoperative observation with respect to testicular position. An ectopic gonad was diagnosed in 15% of the patients, most of whom had a patent processus vaginalis. Gubernacular attachment was in the scrotum in only a third of all cases studied. Undescended testes were softer and smaller than the contralateral gonad in the majority of cases. To our knowledge ours is the first study to correlate preoperative and intraoperative findings in cases of undescended testis. New anatomical as well as morphological and volumetric data are provided.

Age Factors↗

Biliovenous fistula in children after blunt liver trauma: proposal for a simple surgical treatment.

Biliovenous fistula (BVF) with subsequent leakage of bile into the venous system is a rare but serious complication of blunt liver trauma. Nine cases have been reported since 1975. Surgical therapy is indicated; however, there are still controversies as to which operative method should be applied. Based on experience gained in adult surgery, resection of the BVF together with necrotic liver tissue is also recommended in children. We describe both an organ-saving and technically simple method that was applied in two male patients, 2 and 10 years old, respectively, suffering from BVF. After debridement and tamponade of a necrotic cavity of the liver, drainage was carried out. This prevented bilious leak into the venous system. Based on the case history of these two patients, management of BVF fistula will be described. Pathophysiology of bilhemia and the drainage effect will be discussed in light of a review of the literature.

Biliary Fistula↗

KTP-532 laser ablation of urethral strictures.

In 1988 the KTP-532 laser was used to ablate a series of benign urethral strictures. Rather than using a single incision as in urethrotomy, strictures were treated with 360-degree contact photoradiation. Thirty-one male patients, average age 53.2 years, received thirty-seven treatments; 6 patients underwent a second laser treatment. Stricture etiology was commonly iatrogenic (32%), traumatic (16%), and postgonococcal (10%). Stricture location included mainly bulbar (49%), membranous (20%), and penile (12%) areas. The surgical technique consisted of circumferential ablation, followed by Foley catheter placement (mean, 10 days). Follow-up on 29 of 31 patients ranged from one to sixteen months (mean 9.7). Complete success occurred in 17 patients (59%) who had no further symptoms or instrumentation. Partial success was seen in 6 patients (20.5%) with symptom, but not stricture, recurrence. Six patients (20.5%) failed therapy, requiring additional surgery or regular dilations. No complications were seen. Although longer assessment is required, KTP-532 laser ablation of urethral strictures appears efficacious.

Follow-Up Studies↗