Biomedical subjects
Publications and source records attributed to D T Wilcox.
Failure to detect a normally filled fetal bladder on ultrasound scanning can imply the presence of a serious urogenital abnormality. Detailed scanning may elicit the underlying pathology, but there may be complex anomalies present, the full extent of which often has to await the results of postnatal investigations. Management in a combined paediatric urology:fetal medicine clinic is recommended.
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The development of antenatal ultrasonography and the detection of fetal uropathies has created a new field for paediatric urologists. The embryology and physiology of the fetal renal tract is still poorly understood. It is, however, recognised that delays in the maturation of the renal system can lead to transient dilatation of the urinary tract. Structural and functional anomalies can also result in dilatation, but these are permanent. The ability to distinguish between the transient and permanent impairments to urine flow represents a diagnostic challenge. This review article discusses the pathophysiology of urine flow impairment, antenatal detection and the postnatal management of the common congenital uropathies.
The authors report the case of a 12-year-old boy with cystic fibrosis, in whom a percutaneous endoscopic gastrostomy device migrated into the jejunum, forming a gastrojejunal fistula.
PURPOSE: The aim of this study was to review the need for regular endoscopic biopsy of Barrett's esophagus in children. METHODS: This was a retrospective case-notes review of 38 children with Barrett's esophagus treated between January 1982 and August 1997. The mean age at diagnosis was 6.3 years (range, 1 to 15 years). All had gastroesophageal reflux at diagnosis. Two patients were treated medically, and 36 underwent antireflux surgery (32 Nissen fundoplication, four Thal procedures). At follow-up, for a mean of 43 months (range, 0 to 13 years), 25 underwent repeated endoscopy and biopsies, two underwent further surgery and biopsies, and four underwent endoscopy only. Seven have had follow-up at their referring hospital. RESULTS: In the 27 patients who underwent rebiopsy, there was continued evidence of Barrett's esophagus in 15. There was reversion to normal tissue in 10 patients, and mild esophagitis was present in two. There was no evidence of any dysplastic or malignant change in any patient. All cases that reverted to normal esophagus or mild esophagitis had previously undergone a Nissen fundoplication. CONCLUSIONS: Dysplastic degeneration and malignant change did not occur in any of the authors' patients. In addition, 12 patients with Barrett's esophagus reverted to normal. The authors therefore question whether regular endoscopic surveillance is necessary in children under 16 years of age.
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A new variation of open gastrostomy placement is described in which a Stamm gastrostomy is performed via a supraumbilical incision around the circumference of the umbilicus. Access to the stomach is good and the cosmetic result is superior to that obtained with the conventional incision.
The lithotomy position is used in pediatric surgery when it is necessary to have simultaneous access to the abdomen and perineum. At the authors' institution, after a prolonged period in the lithotomy position, significant lower limb complications developed in four patients. Two patients with Hirschsprung's disease underwent a redo Duhamel procedure, one had an anorectal leiomyoma excised, and one had an ileoanal anastomosis for ulcerative colitis. Sciatic nerve injury developed in two patients and deep venous thrombosis and bilateral compartment syndrome resulting in myonecrosis developed in one each. There is an association between operations that require prolonged lithotomy position and the development of postoperative neurovascular complications. By placing the child in the lithotomy position only when access to the perineum is required, these significant injuries may be avoided.
PURPOSE: The aim of this study was to assess the results of the Malone (antegrade colonic enema) procedure for fecal incontinence. METHODS: By a retrospective review of patients treated between 1990 and 1996 in a tertiary referral center, 36 patients were treated with a Malone procedure. Age at operation was 8.3 (range, 3 to 14) years, the mean period of follow-up was 39 (range, 9 to 72) months. The indication was fecal soiling in 35 and chronic constipation in one. The underlying diagnosis was an anorectal anomaly in the majority of patients. The appendix was used in 30 patients and a cecal flap in six, and a submucosal antireflux procedure was also performed in 10. In 35 patients, a circular stoma was created and in one a V flap was used. Antegrade colonic enemas were performed daily in 10, alternate days in 23, and in three patients the stoma was no longer used. Enemas were performed with a 10F catheter using a mixture of phosphate enema (or liquorice) and saline. RESULTS: Fecal soiling was completely controlled in 28 patients, and eight children soiled more than once a week. Complications occurred in 15 patients; the main problem was stenosis of the conduit, which occurred in 9 of 30 appendiceal stomas and three of six cecal stomas. Stomal stenosis was treated with surgical revision in eight patients. Additional complications were reflux through the stoma (n=2), pain on catheterisation (n=1), and small bowel obstruction (n=1). In one patient the Malone has been converted to a colostomy. CONCLUSIONS: The Malone procedure is a simple technique that can effectively control fecal incontinence in the majority of cases. It appeared to be better in older children. Stomal stenosis is a frequently encountered problem that may require surgical revision.
BACKGROUND: A repeat pull-through for Hirschsprung's disease is undertaken when the original procedure has failed. The reasons for failure include a retained aganglionic segment, stricture, fistula, or persisting incontinence. METHODS: All patients who underwent a redo pull-through from 1982 through 1996 were included. Twenty-two patients were underwent surgery; 20 notes were reviewed. The mean age at operation was 6 years (range, 1 to 13). Mean time between operations was 5.1 years (range, 0.5 to 13). RESULTS: Postoperative complications occurred in five patients. These included wound infection, recurrent rectal septum, division of a vas, compartment syndrome, and an anastomotic stricture. Follow-up was for 6.5 years (range, 0.2 to 12). The mean number of bowel movements was 2.2 per day (range, 1 to 6). Fourteen (of 19) patients were continent or soiling less than once per week. Soiling occurred more than once a week in four, but two of these were incontinent after the first operation. One has a colostomy. CONCLUSION: A repeat pull-through is a worthwhile procedure when dealing with an anatomic problem such as retained Hirschsprung's disease, stenosis, or fistula.
OBJECTIVES: To review the urological abnormalities in conjoined twins, a rare congenital abnormality arising from an incomplete separation of a pair of monozygotic twins, and who can be joined from the head to the pelvis with varying degrees of union. PATIENTS AND METHODS: The records and urological anomalies of conjoined twins presenting from 1985 to 1995 were reviewed retrospectively and the management and surgical reconstruction described. RESULTS: Seven of 11 sets of conjoined twins underwent surgical exploration. Complex urological problems occurred in all twins with omphalo-ischiopagus. The urological anomalies were: Set 1; both twins had one lateral crossed fused renal ectopia with uncrossed ureters draining into a single bladder with one urethra. They had one set of genitalia. Each twin received one crossed fused ectopic kidney, half the bladder and genitalia, with one retaining the urethra and one a vesicostomy. Set 2; these twins had four kidneys (two lateral and two central that were fused), four ureters (two of which crossed) and these drained into two sagittal bladders that emptied by one urethra associated with a single set of external genitalia containing three corpora cavernosa. At separation, the crossed ureters were re-routed, each received a bladder and the genitalia were split. Set 3; there were four kidneys with two crossed ureters draining into two collateral bladders, there was a partial duplication of the urethra and a single hypospadiac penis. After separation, each had kidneys draining into a bladder, one received the urethra and external genitalia, and one a urostomy. CONCLUSION: Complex urological problems occur in conjoined twins, although restricted to those with pelvic fusion (ischiopagus). Assessment and treatment needs to be individualised and overseen by a surgeon with experience in complex urology.
Pulmonary hypoplasia and pulmonary hypertension are factors limiting the survival of infants with congenital diaphragmatic hernia (CDH). A reduction in the number of pre-acinar pulmonary vessels and increased muscularization are the structural lesions implicated as causes of irreversible hypoxemia. Whether there is a reduction in the number of air-blood barriers, which represent the capillary surface area of the lung involved in gas exchange, is unknown. We sought to determine if the lungs of CDH lambs have: (1) a reduction in total capillary surface area proportionate to the reduction in the total alveolar surface area of the lung; and/or (2) a disproportionate reduction in the number of capillaries (air blood barriers) within each acinus. The latter measurement was determined by calculating the capillary load which we defined as the number of air blood barriers/unit of surface density. At 80 d gestation (pseudoglandular period), a diaphragmatic hernia was created surgically in one lamb fetus of a twin gestation. At term, the fetuses were removed, the chests opened and the lungs fixed by a tracheal infusion of 1.5% glutaraldehyde at 25 cm of water pressure. Tissues from the lower lobes were examined by light and electron microscopy. Using computerized interactive morphometry, alveolar and capillary surface area, and capillary load were determined by intersection and point counting for the right and left lungs. The data show that the total alveolar surface area of the left CDH and control lungs were 1.8 +/- 0.8 m2 and 6.1 +/- 1.1 m2 (p < 0.01), respectively, and for the right CDH and control lungs 2.5 m2 +/- 0.1 and 11.2 +/- 1.9 m2 (p < 0.01), respectively. The total capillary surface area for the left CDH and control lungs were 0.7 +/- 0.3 m2 and 2.8 +/- 1.2 m2 (p < 0.05), respectively, and for the right CDH and control lungs 0.9 +/- 0.3 m2 and 3.8 +/- 1.5 m2 (p < 0.05), respectively. The capillary load was not statistically different. These findings demonstrate that the lungs in CDH are deficiently vascularized at the alveolar surface due to a reduction in the total alveolar surface area. Each acinus contains the same number of air blood barriers per unit of alveolar surface area indicating a normal acinar composition.
INTRODUCTION: Traditionally Hirschsprung's disease has been treated by a three-stage procedure. This approach has been associated with stoma problems, three episodes of hospitalization, and a theoretical disadvantage, because of the delayed passage of feces via the rectum, in controlling defecation. The aim of this study was to assess the results of one-stage neonatal pull-through for the treatment of Hirschsprung's disease. METHODS: This was a 10-year retrospective review of all patients treated with a neonatal pull-through for Hirschsprung's disease. Information was obtained from the patients' records. All descriptive data are expressed as mean +/- standard deviation. RESULTS: Fifty-one patients were treated in the 10-year period studied. The mean gestational age was 39.6 +/- 1.7 weeks, weight was 3.3 +/- 0.54 kg, with 76% being boys. The surgery was performed at 10.3 +/- 5.8 days of age. A Swenson procedure was carried out in 38 patients and a Soave in 13. The extent of the disease was rectosigmoid in 39, and the remaining were sigmoid, descending colon, and total colonic. The mean length of the surgery was 181 +/- 40 minutes. Blood transfusions were required in 13 patients with a mean volume of 55 mL given. The complications observed were: an anastomotic leak in two cases, requiring laparotomy and colostomy (both in patients with Down's syndrome), and wound infection in three cases. There were two delayed deaths both associated with other congenital anomalies. In total, three patients required further surgery; two had colostomies, one requiring a redo pull-through and one had an ileostomy for enterocolitis. The total length of hospital stay was 20.4 +/- 11.2 days. Mean follow-up was 3.73 +/- 2.9 years. In 21 patients the follow-up was greater than 4 years and in this group none were totally incontinent. Four patients complained of soiling at least once a week, and 17 had normal bowel control. CONCLUSION: Hirschsprung's disease can be successfully treated in the neonatal period with a one-stage pull-through. The short- and long-term results are as good as those with the three-stage procedure, with the child usually benefitting by not having a stoma and a shorter hospital stay.
PURPOSE: In congenital diaphragmatic hernia (CDH) there is immature lung development with a resulting clinical picture of pulmonary hypoplasia, surfactant deficiency, and pulmonary hypertension. Pulmonary hypoplasia and surfactant deficiency both have been successfully treated using partial liquid ventilation (PLV). Pulmonary hypertension associated with CDH has proven difficult to treat, but inhaled nitric oxide, which is a potent highly selective pulmonary vasodilator, may have potential. The aim of this study was to assess PLV in CDH and to document the effect of nitric oxide when administered through perfluorocarbon. METHODS: This study using the lamb CDH model consisted of two groups; a conventional mechanically ventilated (CMV) group and a PLV group. At 1 and 3 hours, nitric oxide (80 ppm) was given for 15 minutes. Data collected included blood gases, pulmonary function tests, pulmonary and systemic blood pressure. RESULTS: After 30 minutes of ventilation, blood gases in the PLV group were all significantly improved (P < .001): pH, CMV 6.92 +/- 0.15 versus PLV 7.24 +/- 0.11; P(CO2), CMV 139 +/- 26 mmHg versus PLV 52 +/- 11 mmHg; P(O2), CMV 26 +/- 15 mmHg versus PLV 184 +/- 60 mmHg. In addition, there was a significant increase in dynamic compliance and a reduction in pulmonary hypertension. Nitric oxide was only efficacious in the PLV group, causing a further increase in oxygenation and a decrease in pulmonary hypertension. These effects were reversed when the nitric oxide was stopped. CONCLUSION: This study shows that PLV both improves gas exchange and pulmonary mechanics in CDH and allows the effective delivery of nitric oxide to reduce the pulmonary hypertension associated with CDH.