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Biomedical subjects

V M Wright

Publications and source records attributed to V M Wright.

At least 19 recordsLinked to original sources

Meconium ileus secondary to cystic fibrosis. The East London experience.

Meconium ileus (MI) affects 15% of neonates with cystic fibrosis (CF). The authors reviewed the management and outcome of 51 neonates presenting to a single institution between 1976 and 1995 with MI secondary to CF. Clinical presentation included abdominal distension (96%), bilious vomiting (49%), and delayed passage of meconium (36%). A family history of CF was present in 4 cases (8%). Twenty-three neonates presented with MI and evidence of volvulus, atresia, or perforation (complicated MI). Of these, 16 underwent stoma formation, 1 appendicectomy, and 6 resection with primary anastomosis. Twenty-eight neonates presented with uncomplicated MI. Of these, 11 were managed non-operatively by Gastrografin enema (10) or enteral N-acetylcysteine (1). The remainder required stoma formation (15) or bowel resection with primary anastomosis (2). Early postoperative complications occurred in 2 neonates (4%). In this hospital the 1-year survival for this condition has increased from 49% (1953-1970) to 98% (1976-1995) irrespective of the surgical procedure performed or the presence of volvulus, atresia, or perforation. In our experience, bowel resection with primary anastomosis is as safe as stoma formation and is associated with a reduced length of initial hospital stay.

Anastomosis, Surgical

Pneumatic intussusception reduction via a colostomy: case report.

Intussusception is uncommon in infants with a colostomy. The authors present the first documented case of intussusception in an infant with a colostomy in which pneumatic reduction was achieved via the stomal opening. The inflated balloon of a Foley catheter was plugged against the stoma at skin level and a tight seal was obtained. Successful reduction was achieved without any adverse effects to the patient.

Catheterization

Double duodenal atresia/stenosis: a report of four cases.

Four neonates with double duodenal atresia/stenosis are described. Preoperative plain radiographs in two patients demonstrated atypical appearances suggestive of complex pathology. Cystic dilatation of the second part of the duodenum was observed at laparotomy in two patients and in one of these the "cyst" was palpable preoperatively, causing diagnostic confusion. All four patients underwent successful surgery but one infant with Down's syndrome subsequently died of congenital heart disease.

Abnormalities, Multiple

The importance of serial biophysical assessment of fetal wellbeing in gastroschisis.

OBJECTIVE: To review antenatal and intrapartum assessment of pregnancies complicated by gastroschisis. DESIGN: Retrospective descriptive study. SETTING: University College Hospital, London. SUBJECTS: 24 consecutive cases of gastroschisis between 1986 and 1991. RESULTS: The gestational age at sonographic diagnosis was 20.3 weeks (SD 6.77) and at birth was 36.5 weeks (SD 2.06). There were 21 live births, all with good surgical outcome. There were 16 vaginal deliveries and eight caesarean sections. The elective sections were for oligohydramnios and dilated bowel (1) and clinically suspected growth retardation (1); the intrapartum caesarean sections were for fetal distress (4) and premature breech presentation (2). There were six with dilated gut on ultrasound; one of these ended in a stillbirth. There was a significant association between gut dilatation and caesarean section for fetal distress (P = 0.004). There was also a significant association between meconium staining and fetal distress (P = 0.021). Of these babies, 46% were < or = third centile for corrected birth weight. CONCLUSIONS: While half of the babies with gastroschisis were small for gestational age at birth, reliable antenatal prediction of birth weight is difficult. Gut dilatation may be an indicator of either antenatal or intrapartum fetal distress, but does not correlate with poor neonatal surgical outcome. We suggest close antenatal surveillance of fetal wellbeing in all cases of gastroschisis because, in addition to growth retardation, many show some evidence of fetal distress and 12.5% end in stillbirth, even when appropriately grown.

Abdominal Muscles

Complications of endorectal pull-through for Hirschsprung's disease.

In 10 years, 57 infants with Hirschsprung's disease underwent endorectal pull-through (ERPT). Postoperatively, 3 patients died. Of the 44 survivors with an intact endorectal anastomosis aged more than 3 years, 23 (52%) had an excellent result, and of the 28 children more than 5 years old, 23 (82%) had a satisfactory result. Of the 53 known survivors of all ages, 18% suffered from diarrhea with intermittent incontinence and 5 (9.4%) had undergone a Duhamel procedure within 4 years of ERPT.

Anastomosis, Surgical

Controversies in the management of gastroschisis: a study of 40 patients.

Forty infants with gastroschisis were referred to two paediatric surgeons during a 13 year period. Overall survival was 90%. Nine patients were transferred in utero and 31 were referred postnatally. Birth weights, gestational ages, and Apgar scores were similar for both groups. Primary closure of the defect was successfully achieved in seven (78%) patients in the prenatally transferred group compared with 17 (55%) in the postnatal group. Significantly less postoperative assisted ventilation, and a trend in favour of early discharge home, were noted after prenatal transfer. Problems arising during postnatal transfer may have contributed to these differences. No major differences resulting from the mode of delivery were identified. Patients treated by primary closure fared significantly better than those undergoing staged repairs with prosthetic material. Prospective randomised studies are required to confirm these findings.

Abdominal Muscles

Results of surgical treatment of neonatal necrotizing enterocolitis.

In 6 years, 74 neonates were treated for necrotizing enterocolitis, 40 (54 per cent) of whom underwent operation. The early postoperative mortality rate was 23 per cent and there was one late death. Of the 30 postoperative survivors, two have short bowel syndrome, two have chronic respiratory disease, two are partially sighted, and five (17 per cent) have severe developmental delay. Only 15 (50 per cent) survivors enjoy good health.

Abnormalities, Multiple

Fetal diaphragmatic hernia: the value of fetal echocardiography in the prediction of postnatal outcome.

Nineteen pregnancies complicated by fetal congenital diaphragmatic hernia (CDH) referred for fetal echocardiography have been reviewed. Congenital heart disease was diagnosed prenatally in three fetuses; in one of these the pregnancy was terminated, the two other infants died in the neonatal period. The presence of the fetal stomach within the thorax or a hernia/heart area ratio greater than 1.7 was associated with a large diaphragmatic defect. Polyhydramnios was not associated with a poor postnatal outcome. Evidence of cardiac ventricular disproportion before 24 weeks gestation in isolated CDH was associated with 100% mortality. Development of ventricular disproportion during the third trimester was associated with a survival rate of 75%. In three fetuses, all of whom survived, no ventricular disproportion was detected during the third trimester examination.

Echocardiography

Oesophageal atresia.

Fifty years ago this congenital anomaly was incompatible with survival. Advances in neonatal intensive care, anaesthesia and surgical techniques permit successful correction in the vast majority of patients. Death in a patient with oesophageal atresia is now rarely attributable to that anomaly, but occurs in the very premature and those babies with other associated major anomalies.

Anastomosis, Surgical

Clostridium difficile and acute enterocolitis.

Clostridium difficile belonging to groups not normally detected in infancy was the only potential pathogen detected in the stools of two infants with severe enterocolitis. Further information regarding the virulence of this organism was obtained by use of a recently introduced typing scheme.

Clostridium

A new surgical approach to duplications of the intestine.

Two unusual intestinal duplications are described. In each case the mesenteric duplication had a separate muscular coat from the normal bowel permitting complete excision of the duplication without interference with the blood supply to the normal intestine.

Female

Chronic granulomatous disease mimicking Crohn's disease.

A 34-month-old boy with intermittent diarrhoea and abdominal distension from 2 months of age, a chronic microabscess of the cheek, gastric antral narrowing, and perianal abscesses containing granulomata was found at colonscopy to have extensive, noncaseating, submucosal ileal and colonic granulomata. He was initially thought to have Crohn's disease, but then developed a cervical abscess, and a diagnosis of chronic granulomatous disease was established. This is an important, although rare, differential diagnosis of chronic inflammatory bowel disease in childhood.

Abscess

Symptomatic vitamin E deficiency in cystic fibrosis.

A girl with cystic fibrosis who developed a neurological syndrome probably secondary to vitamin E deficiency at the age of 10 years is described. The severity of the deficiency and the early development of neurological features probably result from reduced intraluminal bile salt concentrations in addition to the pancreatic insufficiency.

Child

131I macro-aggregated albumin perfusion scanning and chest radiography in asbestos exposure.

131I macro-aggregated albumin perfusion scanning and conventional radiography were used to study the lungs of 108 men who had been exposed to asbestos dust. The presence or absence of basal crackles was recorded in all cases. Forced vital capacity (FVC) was measured in 94 cases. Underperfusion of the lower zones of the lungs was considered to be present in only 8 of 39 cases with small irregular opacities classified as category 1/0 or higher on the 1971 International Labour Organization classification for pneumoconiosis. In 5 of 69 cases classified in categories less than 1/0, perfusion in the lower zones appeared to be impaired. Details of these cases are given. Underperfusion was more common among men with basal crackles or reduced FVC (less than 80% of predicted value) but this trend was not statistically significant.

Adult