Preoperative enterocolitis is associated with poorer long-term bowel function after Soave-Boley endorectal pull-through for Hirschsprung's disease.

BACKGROUND/PURPOSE: The purpose of the investigation was to apply a semiquantitative scoring system for bowel function to patients who had undergone endorectal pull-through (ERPT) for Hirschsprung's disease (HD) and to use this to analyse the clinical factors relating to functional outcome. METHODS: The case note details of 63 patients undergoing ERPT for HD were reviewed. A questionnaire using a semiquantitative scoring system for 6 aspects of bowel function was sent to parents of 55 patients. The total functional score (TFS) for each patient was calculated, and a statistical analysis was performed to determine which clinical factors were significantly related to bowel functional outcome. RESULTS: Fifty (91%) of the parents returned the questionnaire. TFS was "good" in 45%, "fair" in 33% and "poor" in 22%. There was a statistically significant difference in the scores of those who had preoperative Hirschsprung's associated enterocolitis (HAEC) TFS = 8.0 and those who did not, TFS 12.7 (P <.01) The sex of the patient, length of aganglionic segment, timing of ERPT (early/late), staging of ERPT (1 or 2), presence/absence of anastomotic stricture, and presence or absence of Down's syndrome did not statistically significantly affect TFS. CONCLUSIONS: Preoperative HAEC was the most important factor in relation to functional outcome after ERPT for HD. The reason remains unclear.

Twins with oesophageal atresia and the CHARGE association.

Twins are rarely concordant for oesophageal atresia and tracheo-oesophageal fistula. Such a case is presented and their similar associated anomalies described. The CHARGE association is considered as a possible diagnosis and the aetiological implications considered.

A new case of Myhre syndrome.

Myhre Syndrome is a rare condition associated with mental retardation, short stature, generalized muscle hypertrophy, cardiac defects and a distinct facial appearance. There have only been five reported cases and we now present a sixth, together with a review of the clinical features of this syndrome.

The effect of introducing a dedicated emergency theatre and altering consultant on-call commitments in a large children's hospital.

BACKGROUND: Two recommendations of NCEPOD were the introduction of dedicated emergency theatres and a higher level of consultant involvement in emergency cases. In May 1998 a dedicated emergency theatre was introduced in the Royal Hospital for Sick Children, Glasgow. At the same time the on-call rota of the Consultant Paediatric Surgeons was changed from days on-call to a period of one week, during which elective commitments were cancelled. METHOD: A retrospective audit was conducted for the 12-month periods before and after these changes. Start times of emergency cases and grades of surgeons were obtained from the theatre database. RESULTS: The proportion of emergency operations carried out during on call periods (weekends and weekdays after 5 pm) decreased for general paediatric and neonatal surgery (OR = 0.7, p < 0.01). There was an increase in the proportion of daytime emergency cases performed before 1 pm (OR = 0.28, p < 0.01). The Consultant Paediatric Surgeons were involved in more emergency cases (OR = 0.60, p < 0.01). CONCLUSIONS: The provision of a dedicated emergency theatre increased the proportion of emergency cases performed during normal working hours, particularly before 1 pm. Cancelling elective commitments for Consultant Paediatric Surgeons ensured higher levels of consultant input into daytime emergency cases.

Superior mesenteric artery syndrome in children.

Superior mesenteric artery syndrome is an uncommon clinical condition characterised by features of acute or chronic upper gastrointestinal tract obstruction. It sometimes has particular clinical associations such as weight loss, spinal surgery cast application or adhesions. Associated multiple clinical problems can complicate the picture and delay diagnosis. Diagnosis is usually by barium meal showing compression of the 3rd part of duodenum. Treatment can be conservative or surgical. Surgical treatment consists of division of the ligament of Treitz and derotation of the gut. We review our experience of four children with this syndrome.

Atypical mycobacterial infection in childhood: a "surgical disease'.

Atypical mycobacterial (AMB) infection is an important cause of lymph node enlargement in children. Over the period, January 1992 to December 1993, 17 patients with lymphadenitis because of AMB infection were treated at the Royal Hospital for Sick Children in Glasgow. These case notes of the patients were reviewed. The mean age at presentation was 5.37 years (range 1.5-10.6 years). The patients had a short history (1-11 weeks) of unilateral single focus, usually cervicofacial (16/17), disease. The tuberculin skin test was not helpful in diagnosis (negative in 14/15). Primary excision was curative (11/11). Incision and drainage of an abscess or drainage with partial excision led to chronically discharging sinus in all cases (6/6). AMB were not always seen on staining (11/17) of drained or excised material and cultures were negative in eight cases. The diagnosis in the remaining patients was made on the basis of clinical features and particular histopathological patterns. We are currently assessing the use of polymerase chain reaction (PCR) techniques for making the diagnosis in non-cultured cases.

Pneumatic dilatation in childhood cardio-achalasia.

Three boys with achalasia of the cardia were treated with balloon dilatation, with relief of obstruction and weight gain in all cases. Symptoms recurred in one patient, who then underwent myotomy rather than further dilatation. We recommend dilatation as the primary procedure in childhood achalasia.

Thoracic mesenchymoma of infancy.

Mesenchymoma of the chest wall is rare in infancy and can be easily mistaken for a malignant tumor. It is a distinct pathological entity that presents at birth or during infancy as self-limiting expansile intraosseous lesions involving the body of one or more ribs. Histologically, it is a benign focal overgrowth of normal skeletal elements. Two such cases are presented.

Gastroschisis in the decade of prenatal diagnosis: 1983-1993.

Fifty cases (24 males, 26 female) of gastroschisis were admitted to the Regional Neonatal Surgical Unit at the Royal Hospital for Sick Children in Yorkhill, Glasgow, between January 1983 and October 1993. The mean birthweight was 2.17 kgs and mean gestation was 35.8 weeks. Prenatal diagnosis was made by ultrasound scan in 39 cases (78%) and 33 (66%) were delivered in the Regional Obstetric Unit and Fetal Medicine Centre at the Queen Mother's Hospital, Yorkhill, Glasgow, immediately adjacent to the neonatal surgical unit. Spontaneous vaginal delivery occurred in 23 (46%) but Caesarean section was performed in 27 (54%). Sixteen Caesarean sections were emergencies and 11 elective (5 on account of dilated bowel loops). Associated bowel problems were noted in 11 (22%)--atresia (5), stenosis (2), ischaemia (1), duodenal perforation (1), ileal perforation (1) and jejunal band obstruction (1). Primary closure was achieved in 42 (84%) and there were 5 deaths (10%). Prenatal diagnosis and mode of delivery showed no direct correlation with mortality (10%) in this series. Emergency Caesarean section was necessary for fetal indications in 28%. The prediction of compromised or damaged intestine by prenatal ultrasound was unreliable and the majority of cases of atresia and stenosis escaped detection. However, it is concluded that prenatal diagnosis and delivery in a regional centre offers the best outcome for the fetus. Further study is needed to define "at risk" pregnancies and fetuses and to determine the role of intervention.

Acute pancreatitis in children: a 15-year review.

AIM: To review the aetiology, presentation, diagnosis, management, and sequelae of acute pancreatitis (AP) in children and to assess the relevance of prognostic criteria used to assess severity in adult AP. METHOD: A retrospective review (1978 through 1992) of childhood AP managed in three Scottish paediatric centres. RESULTS: Forty-nine cases of AP (34 male, 15 female) were reviewed (mean age, 7.1 years). The presenting features were abdominal pain and vomiting (80%), abdominal tenderness (73%), and parotid enlargement (22%); back pain was rare (8%). Ultrasound scan (US) findings were abnormal for 24 of 34 patients. Forty-one (82%) were managed conservatively. Six (12%) underwent early laparotomy; three (6%) underwent laparotomy after trauma. Five required subsequent definitive surgery. One patient died. Thirty-five (70%) suffered no sequelae, and five (10%) had further acute pancreatitis. Only half of the eight Imrie prognostic criteria had been recorded in these patients; only three were judged to have severe AP by the criteria. Other "clinically severe" cases were not identified. CONCLUSION: The most common causes of AP were mumps (39%) and trauma (14%); in 12, no cause was identified. US was the most useful imaging tool. The Imrie criteria were of doubtful value but warrant further prospective analysis and possible modification for children.

Facial clefts in the west of Scotland in the period 1980-1984: epidemiology and genetic diagnoses.

Two hundred and eighty six cases of cleft lip, cleft palate, or both were identified in a study attempting complete ascertainment of babies with facial clefts born to women resident in the west of Scotland in a five year period beginning 1 January 1980. The total birth prevalence (TBP) of these defects over this period was 1.53 per 1000. The TBP for cleft lip with or without cleft palate (CL[P] was 0.74 per 1000 and for cleft palate (CP) was 0.79 per 1000; 26% of CL[P] and 39.5% of CP cases had one or more major congenital anomaly associated with their facial cleft and in over half of these cases a specific genetic or syndrome diagnosis could be made. In comparison to previous European reports this study shows a high incidence of associated abnormalities and a remarkably low ratio of CL[P]:CP cases.

Prenatal diagnosis and management of anterior abdominal wall defects in the west of Scotland.

An attempt was made to identify all the cases of abdominal wall defects occurring in the West of Scotland over a 7-year period to determine the current incidence, prenatal diagnosis, management, and prognosis for fetuses and neonates with abdominal wall defects. Cases were identified because they presented either for prenatal diagnosis, or to the Department of Pathology following termination or spontaneous pregnancy loss, or as neonates to the Neonatal Surgical Department. The incidence of abdominal wall defects was found to be 1 in 2500 births. Exomphalos was diagnosed before birth in 66 per cent of cases, and in 30 per cent of cases it was associated with another major abnormality. There was a 20 per cent intact survival in the cases diagnosed prenatally who had no fetal anomaly and who opted to continue with the pregnancy. Gastroschisis was diagnosed before delivery in 70 per cent of cases, and in the group who continued with the pregnancy there was an intact survival of 77 per cent. Body stalk anomalies were all diagnosed prenatally and terminated. Maternal serum alpha-fetoprotein was elevated in 89 per cent of the cases with exomphalos and in 100 per cent of the cases with gastroschisis and body stalk anomalies in which it was tested.

Prenatal diagnosis of an intra-abdominal sacrococcygeal teratoma.

The prenatal diagnosis of a presacral (type IV) sacrococcygeal teratoma (SCT) is described. The initial ultrasound appearance was suggestive of a lower urinary tract obstruction, but further ultrasonic examination and radiological imaging using contrast medium led to the diagnosis of SCT. This is the first prenatal diagnosis of a totally intra-abdominal SCT.

Pseudomembranous enterocolitis--a potentially lethal disease.

The cases of nine children with pseudomembranous enterocolitis, five of whom died, are reported here. Six had prior antibiotic therapy, and in eight there were other diseases present that were considered to contribute to the development of this disease. Awareness of this condition, which requires aggressive medical and surgical management, is discussed.

Value of antenatal diagnosis of abnormalities of the urinary tract.

OBJECTIVE: To assess the value of antenatal diagnosis of abnormalities of the urinary tract on ultrasonography. DESIGN: Retrospective study. SETTING: Two obstetric units in Glasgow. SUBJECTS: 62 Fetuses in which renal abnormalities were diagnosed on antenatal ultrasonography. INTERVENTIONS: Six fetuses had their bladders aspirated to determine renal function. Fifteen pregnancies were terminated on the basis of the findings on antenatal ultrasonography, and if possible necropsy was performed on the fetuses. In babies who were born alive the final diagnosis was made by postnatal ultrasonography, intravenous urography, radionuclide scanning, cystography, and, in those who died in the early neonatal period, necropsy. Neonates who were referred with a known obstructed kidney had nephrostomy or pyeloplasty. END POINT: Assessment of the value of antenatal diagnosis of renal abnormalities on ultrasonography for babies who had no clinical evidence of disease postnatally. MAIN RESULTS: Eighteen fetuses did not survive birth; the antenatal diagnosis was accurate in all 18. Of the 44 babies born alive, five had normal urinary tracts, in two of whom antenatal ultrasonography had probably indicated a false positive diagnosis. Fourteen babies died during the early neonatal period. Twenty five babies with renal abnormalities were followed up; the antenatal diagnosis was inaccurate for 10 of them, the commonest misdiagnosis being hydronephrosis for multicystic kidney and vice versa, and there was one false positive diagnosis. The initial clinical findings in 14 babies would have led to the early detection of a urological abnormality. In the 30 babies with no clinical evidence of disease the antenatal diagnosis was of definite value in eight, probable value in 15, and marginal value in seven. Overall, an accurate antenatal diagnosis was made in 46 of the 62 cases (74%); in 12 cases renal disease was detected but its specific nature was not determined; and in four cases the diagnosis was misleading. CONCLUSIONS: The overall value of antenatal diagnosis is that it indicates early termination of fetuses with fatal renal disease, prepares parents and medical staff for the likelihood of serious neonatal problems, and shows abnormalities of the urinary tract that may not be detected postnatally.

Diaphragmatic hernia presenting in utero as a unilateral hydrothorax.

A right-sided diaphragmatic hernia presented as a fetal hydrothorax. The fluid-filled hernial sac enveloped the lung and presumably prevented serious compression effects. The implications of fetal thoracic anomalies are discussed.

Choledochal cyst: a 33-year review.

We present 13 patients who were seen at Glasgow hospitals over a 33-year period. Only five (38 per cent) were children. Of the eight adults, two had complications of the cyst at presentation, namely biliary cirrhosis and cholangiocarcinoma respectively. Another adult patient developed a carcinoma in the cyst 9 years after internal drainage. The incidence of malignant change in the series was therefore 15 per cent. Only five patients have remained well after surgery: three had complete excision and two internal drainage. Of the eight other patients, one had complete excision, five had internal drainage, one had partial excision and one had cholecystectomy and (years later) sphincterotomy. We believe that excision of the cyst is the treatment of choice.