[Show abstract][Hide abstract] ABSTRACT: Fetal magnetic resonance imaging (fetal MRI) is an important adjunct to antenatal imaging especially when neonatal surgery is contemplated. We report two cases of fetal nuchal tumors, which were diagnosed incidentally on an ultrasound scan and had fetal MRI to aid diagnosis, prognosis, counseling and management planning. In the first case, fetal MRI aided diagnosis and prenatal multidisciplinary management of a cervical teratoma. Tracheal involvement could not be excluded and an ex-utero intrapartum treatment procedure was planned. Postnatal MRI and angiography provided further information prior to surgery. In the second case, fetal MRI assisted thorough counseling following the finding of a cervical lesion thought to be a cervical teratodermoid, a multicystic hygroma or congenital lymphectasia. The parents opted for termination of the pregnancy. Postmortem findings confirmed the extent of involvement of surrounding structures diagnosed prenatally. The mass was found to be a hamartomatous hemangiolymphangioma.
Full-text · Article · Nov 2005 · Ultrasound in Obstetrics and Gynecology
[Show abstract][Hide abstract] ABSTRACT: To describe trends in incidence, associated anomalies, clinical outcomes and sensitivity of prenatal diagnosis for congenital malformations of the diaphragm in the West Midlands Region between 1995 and 2000.
Information was retrieved from a population-based register of major congenital malformations in a health region of England, the West Midlands Congenital Anomaly Register (WMCAR), between 1995 and 2000.
One hundred and sixty-one confirmed cases of congenital malformations of the diaphragm were notified from 396 577 births. This gives an incidence of 4.1 per 10,000 births. After natural losses and terminations, the incidence at birth was 2.9 per 10,000 registered births. For live-born cases, the infant mortality rate was 317 per 1000 births. 47% of the cases had additional structural or chromosomal anomalies; the infant mortality rate for these complex cases was 533 per 1000, an increased relative risk of 2.37 compared with isolated lesions. 66% of the cases were diagnosed prenatally, 51% of isolated lesions and 84% of complex cases. Fourteen prenatally diagnosed cases (12%) were false-positives; however, 11 of these cases had other significant pathology. These 14 cases were not included in the 161 confirmed cases.
Congenital malformations of the diaphragm remain associated with considerable infant mortality. Most cases are now diagnosed before birth and the prognosis is adversely affected by the presence of other structural or chromosomal anomalies. This presents significant challenges for those involved in counselling the parents of affected fetuses.
No preview · Article · Aug 2004 · Prenatal Diagnosis
[Show abstract][Hide abstract] ABSTRACT: The aim of this study was to review the effectiveness of resecting dilated distal bowel in children suffering unmanageable constipation or soiling who have been operated on previously for anorectal malformations.
A retrospective review was performed of 9 children. Each child underwent excision of dilated bowel to leave normal caliber bowel anastomosed by hand to a rectal reservoir at the peritoneal reflection. The documented follow-up was reviewed.
The 9 children had primary surgery for the following anomalies: high (n = 1), intermediate (n = 1), low (n = 3), rectal stenosis (n = 3), and anal stenosis (n = 3), Seven children had persistent fecalomas, and 7 had major problems with soiling. All were on large doses of laxatives, with 5 having regular rectal washouts and 4 having regular enemas. In all radiologic studies there was a prompt change from normal caliber bowel to dilated bowel at the upper limit of the dilatation. The mean age at operation for excision was 4 years, 11 months (range, 11 months to 9 years, 11 months). The mean period of follow-up was 4 years, 7 months (range, 2 years, 3 months to 10 years). Follow-up showed that all children improved. None had major complications. All were having between one and 3 bowel actions per day. Three continued to soil but improved. Of the remaining 6, only 2 required occasional laxatives and had regular spontaneous bowel actions without soiling. No child was having enemas or washouts.
Anterior resection for the treatment of megarectosigmoid is a safe and effective procedure.
No preview · Article · Nov 2002 · Journal of Pediatric Surgery
[Show abstract][Hide abstract] ABSTRACT: A degree of feed intolerance after neonatal abdominal surgery is common but in an otherwise well baby enteral feeding usually is continued at the highest tolerated level. However, the presence of rectal bleeding, pneumatosis intestinalis, or portal vein gas seen on plain abdominal x-rays suggest the possibility of postoperative necrotising enterocolitis. When this happens feedings usually are stopped for 7 to 10 days, and intravenous antibiotics and total parental nutrition are commenced.
The authors report 12 episodes of rectal bleeding and 11 episodes of pneumatosis intestinalis in 3 infants who previously had undergone neonatal abdominal surgery for intestinal malformations. In 7 of these episodes, feedings were neither stopped nor were antibiotics given. At the time of these 7 episodes, the infants were more than 3 kg in weight, had no significant cardiac or respiratory pathology, were all clinically stable, had no evidence of peritonitis, had no thrombocytopenia, and were greater than 37 weeks postconception.
The 3 infants were monitored closely. There were no early or late problems observed attributable to this management.
Carefully selected clinically stable patients that have postoperative pneumatosis intestinalis or exhibit rectal bleeding may be successfully managed by reduced enteral feedings with no antibiotics. J Pediatr Surg 36:1820-1823.
No preview · Article · Jan 2002 · Journal of Pediatric Surgery
[Show abstract][Hide abstract] ABSTRACT: Ectopic ureters present in childhood with symptoms related to an abnormal site or structure (refluxing, obstructed) of the ureteric orifice. The majority drain duplex kidneys. The diagnosis is relatively easy if the poles are functioning or hydronephrotic. Associated malformations are rarely seen and the results of surgery are gratifying. If an ectopic ureter drains a single kidney, it is called a single-system ectopic ureter (SSEU). We reviewed a 15-year experience (1980-1995) with 127 ectopic ureters from our hospital:11 SSEUs in ten consecutive children were managed during this period. Our data lead us to believe that SSEUs are a special subset of ectopic ureters. Diagnosis is often delayed because the ectopic ureter may be associated with a single small, dysplastic, poorly-functioning, non-visualised kidney and the child may be thought to have a contralateral normal "solitary kidney". Associated systemic malformations are common. Residual symptoms of wetting may persist in the early postoperative period. A high degree of suspicion must be maintained for this entity when a child presents with urinary symptoms of wetting or recurrent infection and a "solitary kidney". Early endoscopic examination of the genitourinary tract will clinch the diagnosis in the majority of cases. Residual symptoms of wetting in the postoperative period generally resolve with passage of time.
No preview · Article · Dec 2001 · Pediatric Surgery International
[Show abstract][Hide abstract] ABSTRACT: Gastroschisis (GS) is the commonest abdominal-wall defect in the Western world. The conventional practice has been reduction of the viscera and closure of the abdominal wall as an emergency procedure. The testis is often a part of the prolapsed viscera along with the bowel loops, stomach, fallopian tube, etc. The primary management of prolapsed (PT) (3) and intra-abdominal (5) testes (IAT) in this condition was studied in 16 consecutive male babies with GS, each was managed by simple reposition of the testes and closure of the abdominal wall. The babies were followed up for spontaneous descent of the testes. At 18-month follow-up, all five IAT had descended into the scrotum spontaneously and were palpably normal. Of the three extra-abdominal PT, two had descended into the scrotum and were normal in size and on palpation. One was palpable in the superficial inguinal pouch. Simple reposition of the testes into the abdomen and closure of the abdominal defect is the correct approach for primary management of PT or IAT in a newborn with GS.
No preview · Article · Aug 2001 · Pediatric Surgery International
[Show abstract][Hide abstract] ABSTRACT: Pyloric atresia is an uncommon condition occurring in 1 of 100,000 live births. When occurring in isolation, the clinical course usually is uncomplicated after surgical treatment. However, it may occur in association with other congenital abnormalities. The authors present 5 new cases, 3 of associated abnormalities including 1 of esophageal atresia and 2 of agenesis of the gall bladder and malrotation. Agenesis of the gall bladder has not been described previously in combination with pyloric atresia. The literature has been reviewed and guidelines are suggested for the management.
The case records of 4 neonates who presented to the author's institution between January 1998 and June 1999 and 1 who presented at another center in 1991 were reviewed. A Medline literature search was performed, and guidelines were developed for the management of this condition based on our cases and the literature review.
Patients 1 and 5 had no associated anomalies. Patient 2 had associated esophageal atresia, tracheoesophageal fistula, atrial septal defect, crossed renal ectopia, malrotation, and absent gall bladder. Patient 3 had a rectovestibular fistula, vaginal atresia, atrial septal defect, malrotation absent gallbladder, and absent extrahepatic portal vein. Patient 4 had epidermolysis bullosa. Patients 2 and 5 had unremarkable recoveries, patients 2 and 3 had markedly delayed gastric emptying that responded to cisapride. Patient 3 has portal hypertension and remains under close follow-up. Patient 4 died at 22 days of age of pseudomonas sepsis.
Based on our cases and literature review, we have adopted the following guidelines: (1) All children with pyloric atresia should be screened for multiple anomalies. (2) Delayed gastric emptying should be considered early and may respond to prokinetic agents. (3) Association with Epidermolysis bullosa should not preclude surgical treatment. (4) A skin biopsy specimen should be taken at the time of surgery for electron microscopy if there is a family history of epidermolysis bullosa.
No preview · Article · Sep 2000 · Journal of Pediatric Surgery
[Show abstract][Hide abstract] ABSTRACT: EDITOR—Stone et al have a very different view from ours of abdominal wall defects in our regions (former South East Thames and North West Thames).1 As they acknowledge, the cited rates for abdominal wall defects were derived from sources with different reporting patterns. In particular, the rates quoted for England and Wales were from data from the Office for National Statistics,2 which take no account of terminations for abnormalities diagnosed prenatally, whereas the Glasgow and northern England registers have tried to be comprehensive. Incidence of abdominal wall defects according to former South East Thames and North West Thames registers, 1992-6
No preview · Article · Mar 1999 · BMJ Clinical Research
[Show abstract][Hide abstract] ABSTRACT: Exomphalos affects approximately 3 in 10,000 births and can arise from a number of developmental insults. The clinical outcome is dependent upon the associated structural and chromosomal anomalies and the gestation at delivery. Accurate antenatal ultrasound diagnosis and karyotyping are important and allow informed prenatal and postnatal management decisions to be made. Prenatal care and counselling should be multidisciplinary and information should ideally be given to parents regarding prognosis and outcome based on prospectively collected population-based data.
No preview · Article · Jan 1999 · Prenatal Diagnosis
[Show abstract][Hide abstract] ABSTRACT: Short bowel syndrome has significant morbidity and is potentially lethal especially when intestinal loss is extensive. The pathophysiology of short bowel syndrome, its aetiology, prognosis and our understanding of the mechanisms of adaptation are reviewed. Management by a multi-disciplinary nutritional care team is advocated and should be directed to the maintenance of growth and development, the promotion of intestinal adaptation, the prevention of complications and the establishment of enteral nutrition. The choice of enteral feed, the role of drugs and the use of pro-adaptive nutrients and agents are discussed. Complications including cholestasis and catheter related sepsis are outlined with strategies to reduce them. Finally the roles of secondary surgical interventions including transplantation are discussed.
No preview · Article · Jan 1999 · Baillière s Clinical Gastroenterology
[Show abstract][Hide abstract] ABSTRACT: To assess the efficacy of cisapride in reducing ileus persisting to the tenth postoperative day after neonatal abdominal surgery.
A prospective, randomised, double blind trial comparing rectal cisapride (1.4-2.3 mg/kg/day) with placebo over seven days was undertaken in 33 neonates.
Seven of 12 (58%) patients receiving placebo and eight of 11 (73%) receiving cisapride achieved a first sustained feed during treatment. Of those receiving cisapride, the first sustained feed occurred at 2.3 days (SEM 0.6) compared with 4.7 days (SEM 0.8) with placebo. By the seventh day the mean daily net enteral balance was 69 (SEM 18) ml/kg in the cisapride subgroup and 17 (SEM 8) ml/kg for those receiving placebo. Stool was passed on 6.3 (SEM 0.4) treatment days in the cisapride subgroup compared with 4.1 (SEM 1.0) treatment days in the placebo subgroup.
Cisapride is effective in neonates with a prolonged ileus after abdominal surgery.
Preview · Article · Oct 1997 · Archives of Disease in Childhood - Fetal and Neonatal Edition