Acute pancreatitis in children - morbidity and outcomes at 1 year.

OBJECTIVE: To establish short-term and medium-term complications 1-year postdiagnosis, of acute pancreatitis (AP) in children aged 0-14 years. DESIGN: One-year follow-up of a prospective monthly surveillance of new cases of AP in children under 15 years through the British Paediatric Surveillance Unit (BPSU) from April 2013 to April 2014. SETTING: A monthly surveillance of >3700 consultant paediatricians and paediatric surgeons in the UK and Ireland using the BPSU. PATIENTS: Children aged 0-14 years with a new diagnosis of AP. MAIN OUTCOME MEASURES: The outcomes following AP, including the incidence of complications and comorbidity at diagnosis and at 1 year. RESULTS: Of the 94 new confirmed cases of AP identified in the UK during the study period, 90 cases (96%) were included in the 1-year follow-up. 30 patients (32%) developed further episode(s) of AP. Over one-fifth of patients developed one or more major complication. At initial admission, the most common of these was pancreatic necrosis (n=8, 9%), followed by respiratory failure (n=7, 7%). Reported complications by 1 year were pseudocyst formation (n=9, 10%), diabetes requiring insulin therapy (n=4, 4%) and maldigestion (n=1, 1%). At 1-year postdiagnosis, only 59% of children made a full recovery with no acute or chronic complications or recurrent episodes of AP. Two patients died, indicating a case fatality of ~2.0%. CONCLUSIONS: AP in childhood is associated with significant short-term and medium-term complications and comorbidities including risk of recurrence in approximately a third of cases.

Injury to the common iliac artery during suction rectal biopsy.

A case of injury to the common iliac artery during suction rectal biopsy is described. Factors that might influence the incidence of complications after suction rectal biopsy are considered. Suction rectal biopsy, although invaluable in establishing the diagnosis of Hirschsprung's disease, is a potentially dangerous technique and should not be delegated to an inexperienced operator. In neonates it is unwise to take biopsy specimens more than 4 cm from the anal verge. A posterior approach is likely to be safer than an anterior or lateral one.

Development of a technique for jejunal interposition in long-gap esophageal atresia.

Primary anastomosis is the treatment of choice in esophageal atresia. There remains a small number of infants in whom establishment of primary esophageal continuity fails or is unrealistic due to a long gap. Esophageal reconstruction then necessitates an interposition/transposition procedure. The most widely used tissues are colon and stomach but each has significant limitations. Jejunum is theoretically the ideal esophageal substitute being of appropriate diameter and having good peristaltic activity. Its use in the past has been limited by the precarious blood supply and restricted length which result from the short mesenteric pedicle. We have attempted to resolve these limitations by using a microvascular anastomosis to augment the blood supply to the proximal jejunum in a series of 5 cases (2 pure atresias, 1 esophageal atresia with proximal fistula, and 2 atresias with distal fistula). In a sixth case (atresia with distal fistula) a free jejunal graft was used. In one case initial surgery was confined to cervical esophagostomy and feeding gastrostomy, in the remaining 5 interposition was necessitated by failure of a primary repair. The age at surgery ranged from 8 to 16 months. The development of the technique and outcome in each patient is described. We conclude that a free jejunal graft is preferable to augmenting the native blood supply and intend to continue with this latter technique.

Mortality after an antenatal diagnosis of foetal uropathy.

A longitudinal study identified 987 foetal uropathies over a 13-year period. There were 147 deaths. Forty infants died as a result of a lethal uropathy in the presence of associated congenital anomalies. Sixty-six infants with an isolated uropathy died. There were 4 cot deaths, 2 obstetric related deaths, and 34 deaths caused by associated congenital anomalies. There was 1 termination of pregnancy following a false-positive diagnosis of uropathy. Of the 147 deaths, 34 occurred postnatally, 20 within 24 hours. Twenty-nine infants were spontaneously aborted. There were 78 terminations of pregnancy, 43% occurring after 24 weeks of gestation. There was complete concordance in antenatal and postnatal diagnoses in 113 (77%) cases and incomplete concordance in 19 (13%) cases. There were 14 false-negative diagnoses (9.5%). The relative frequency of different lethal congenital uropathies is detailed. Accurate in utero diagnosis of foetal uropathy and hence prediction of outcome is possible. The relatively late gestational age at time of diagnosis remains a constraint when fatal malformations would otherwise prompt termination of pregnancy.

Is delayed surgery really better for congenital diaphragmatic hernia?: a prospective randomized clinical trial.

Delayed surgery has become widely accepted in the management of congenital diaphragmatic hernia after comparing outcomes only with historical retrospective controls. It was the aim of this study to compare early and delayed hernia repair in a randomized prospective clinical trial. Fifty-four infants were randomized to receive either early repair (within 4 hours of admission) or delayed repair (more than 24 hours after birth). The survival rate was higher for the delayed group (57% v 46%), but the difference was not significant (difference: -11; 95% confidence limits: -37.5, 15.5). There were no significant differences between the two groups with respect to length of hospital stay, ventilator dependency, or survival time. Recorded preoperative risk factors were similar for the two groups. Eight infants in the delayed repair group died without having undergone surgery. The optimum time for surgery still needs clarification.

Juvenile xanthogranuloma with extra-cutaneous lesions--a case report.

Juvenile xanthogranuloma (JXG) is a rare affliction of early childhood comprising cutaneous and deep-seated lesions. Accurate diagnosis is important as the condition is self-limiting with spontaneous regression over a period of months. A case of congenital JXG is reported and the literature briefly reviewed.

Risk of neoplasia and malignancy in "dominant" thyroid swellings.

OBJECTIVE: To determine the risk of neoplasia and malignancy in "dominant" thyroid swellings. DESIGN: Prospective analysis during six years. SETTING: Thyroid clinic serving the Grampian region. PATIENTS: 574 consecutive patients presenting with a discrete thyroid swelling, of whom 179 (31%) were classified clinically as having a dominant area of enlargement within a multinodular gland. RESULTS: After clinical and cytological assessment 77 dominant swellings were excised. Of the excised swellings, 45 were non-neoplastic and 32 neoplastic, including 11 malignant lesions. The minimum incidence of neoplasia and malignancy in all 179 dominant swellings was therefore 18% and 6% respectively. CONCLUSION: Dominant thyroid swellings should be regarded with greater clinical suspicion than has been traditional.

Management of isolated thyroid swellings: a prospective six year study of fine needle aspiration cytology in diagnosis.

OBJECTIVE: To audit the accuracy and impact on the frequency of operation of fine needle aspiration cytology of isolated thyroid swellings. DESIGN: Prospective analysis over six years of cytological predictions compared with histological findings. SETTING: Thyroid clinic serving the Grampian region. PATIENTS: 395 Consecutive patients presenting with an isolated thyroid swelling, 307 of whom underwent surgical excision. Analysis was confined to a subgroup of 283 patients with satisfactory aspirates who were operated on. RESULTS: The positive predictive value of aspiration cytology for detecting malignant disease was 100% and the sensitivity 83%. The sensitivity for the detection of neoplasia (frank malignancy together with follicular adenomas) was 76%. The specificity was 58% and the overall accuracy 69%. Recalculation of data in previous papers with strict criteria showed the accuracy of aspiration cytology to be variable and lower than is widely accepted. Since the introduction of aspiration cytology 21% fewer operations for isolated thyroid swellings have been performed. CONCLUSIONS: As a basis of selection for surgical excision of isolated thyroid swellings according to prediction of neoplasia fine needle aspiration cytology is less reliable than is widely accepted. It is an adjunct to management rather than a definitive test, and negative cytological results do not exclude neoplastic disease. Further study should take account of the implications of repeated clinic attendances for review and aspiration as these may culminate in delayed surgical treatment.

Bilateral inflamed paratubal cysts.

Paramesonephric duct remnants are an infrequent cause of abdominal symptoms in childhood. Preoperative diagnosis is often difficult and diagnosis is usually made at surgery. We report a rare presentation of an acute abdomen in a child with bilateral inflamed fimbrial cysts. Ultrasound revealed the presence of a multicystic lesion behind bladder. It was only at laparotomy the diagnosis of bilateral inflamed fimbrial cysts was establsihed. These were excised and the child made an uneventful post operative recovery.

Watson fundoplication in children: a comparative study with Nissen fundoplication.

BACKGROUND: Nissen fundoplication is the gold standard antireflux procedure in children. In 1996, one pediatric surgeon adopted the anterior fundoplication described by Watson in 1991. This procedure is reported to achieve good reflux control while permitting burping, active vomiting, and reducing gas bloat. An audit project was undertaken to compare the clinical outcome of children undergoing Nissen and Watson fundoplication. METHODS: The case notes of 144 children undergoing open fundoplication between February 1995 and February 2002 were reviewed retrospectively. RESULTS: Results of 72 boys and 59 girls comprising 76 Nissen and 55 Watson fundoplications were assessed. In each group, one death occurred within 1 month of operation. Chest infections occurred in 6.6% (Nissen) and 1.8% (Watson), and wound infections in 2.6% and 1.8%, respectively. Dysphagia was recorded in 7.9% of Nissen and 1.8% of Watson fundoplications. Follow-up data were analyzed in 70 children with Nissen and 48 children with Watson fundoplication. When overall clinical outcome was assessed for those patients with a minimum follow-up of 1 year, 85.1% Nissen and 88.2% Watson were judged good/excellent; 14.9% Nissen and 11.8% Watson were judged poor/bad. CONCLUSION: Watson fundoplication can safely be performed in children with comparable clinical outcome to Nissen fundoplication.

Idiopathic fibrosing pancreatitis and spontaneous resolution of pancreatic masses in children.

A symptomatic pancreatic mass poses both a diagnostic and therapeutic conundrum. First, benign conditions may mimic malignancy and second, the management of benign lesions has not been well defined. We reviewed four such cases and discuss the management of juvenile idiopathic fibrosing pancreatitis in the light of these. In four children with symptomatic pancreatic masses/enlargement (three with jaundice) all resolved spontaneously without definitive intervention. Histopathology, obtained in two cases, revealed a fibrotic chronic inflammatory reaction. Pancreatic masses in children may not be malignant. Resolution of symptoms including obstructive jaundice and a mass secondary to juvenile idiopathic fibrosing pancreatitis may occur spontaneously, avoiding the need for resection or definitive drainage procedures.

Prenatal diagnosis of echogenic lung: evolution and outcome.

OBJECTIVES: Despite the feasibility of detecting lung lesions by antenatal ultrasound, there are problems in correlating the prenatal diagnosis with the final histology and in predicting the outcome. In order to better describe these factors, we reviewed the outcome of fetuses that had been diagnosed with echogenic lung in a referral fetal medicine unit. METHODS: We searched the database of a tertiary fetal medicine unit for all cases of fetal echogenic lung seen since 1994 and studied the maternal and neonatal records found. RESULTS: There were 48 cases of echogenic lung diagnosed at a median gestational age of 21 (range, 19-29) weeks, including 43 (90%) cases of congenital cystic adenomatoid malformation (CCAM) and 5 (10%) of pulmonary sequestration (PS). The evolution of the fetal abnormality after diagnosis was: in 22 (45.8%) cases the lesion disappeared; in 17 (35.5%) cases the lesion remained stable and six (12.5%) cases became severe. Three (6%) women underwent termination of pregnancy. The lesions were equally distributed between the two sides of the thorax. Mediastinal shift was associated with a threefold increase in the possibility of clinical deterioration (17% vs. 5%), and the disappearance of the lesion was twice as likely to occur when the lesion was classified as microcystic as when it was macrocystic (67% vs. 36%). Features of hydrops were found in 9 (21%) fetuses and in six (13%) cases progressed and resulted in intrauterine or neonatal death. Sixty-four percent of cases with lesions that disappeared during the pregnancy had an abnormal computed tomography (CT) scan, and the prenatal diagnosis correlated with histology in 36% of these cases. Of the cases in which the lesions remained stable, 70.5% had an abnormal CT scan and the prenatal diagnosis correlated with the histology in 67% of the cases. CONCLUSIONS: Prenatally diagnosed echogenic lung has a good prognosis in the absence of hydrops. The ability to correctly assess echogenic lung lesions and the need for surgery by prenatal ultrasound is limited.

Oesophageal substitution by jejunal free graft: follow-up data and an evaluation.

We have developed a new technique for oesophageal substitution using a jejunal free graft and now present a 3-5 year follow-up study of five infants who had this procedure performed for long gap oesophageal atresia. Swallowing is excellent in two, good in two and fair in one. We conclude that jejunal interposition, though technically difficult, can produce excellent results and we particularly recommend this technique, when the oesophageal substitute has to reach high in the thorax or to the neck.

Pneumatic dilatation for childhood achalasia.

Treatment of achalasia by pneumatic balloon dilatation (PBD) is well established in adults. Due to limited experience and the rarity of the condition in children, there are relatively few reports in the paediatric literature. Although PBD has been reported as a primary method of treatment, there are no reports of secondary PBD for childhood achalasia. Between 1995 and 1999, five patients underwent treatment for achalasia (age: 9-14 years, M:F = 4:1). The presenting symptoms were dysphagia (5). vomiting episodes (2), aspiration (1), food-bolus obstruction (1), and failure to thrive (1). In all patients a barium swallow and manometry were used to confirm the diagnosis. Three underwent primary PBD. Two who had previously undergone surgical myotomy underwent secondary PBD for recurrence of symptoms. Dilatation was performed using a 35-mm balloon with the child under general anaesthesia. Technical success was defined as demonstration of a waist under screening at lower pressures followed by abolition of the waist at higher pressures. In addition to reviewing our results, a systematic review of the literature was performed (Medline, Cochrane Library, Pubmed, Embase). Three patients (primary dilatation) showed excellent improvement after a single dilatation. In two cases (secondary dilatation) three and five attempts were required. No complications were encountered. The mean follow-up period was 2 years (1-3.5 years) and four patients remained asymptomatic, an overall success rate of 80%. The literature review revealed similar good results in most of the recent reports. Thus, PBD as a primary treatment for childhood achalasia has a success rate of 70%-90% with minimal side effects, short hospital stay, and good patient acceptability over an operation. We have also established the usefulness of this method as a secondary treatment when symptoms recur after surgery.

Anorectal malformation with recto-perineal fistula: case report and clues to diagnosis.

Rectoperineal fistula is an unusual form of anorectal malformation in a boy. The authors report on a patient with this anomaly and the implications in terms of difficulty in diagnosis and neonatal management.

Small-bowel continuity: a crucial factor in determining survival in gastroschisis.

A retrospective analysis of a series of 63 cases of gastroschisis managed over an 11-year period distinguished a single statistically significant prognostic factor. There were 6 (9.5%) deaths, of which 4 occurred in the 8 infants with small-bowel atresia/stenosis (P < 0.005, Fisher's exact test). One died at 48 h and the remaining 3 of liver disease related to total parenteral nutrition. Of the 4 survivors, 1 developed a late biliary stricture necessitating hepaticoenterostomy but is alive and well aged 4 years. The remaining 3, following initially prolonged hospitalisations and multiple operations, are alive and well after 2, 4 and 7 years. In 3 patients the atresia was not detected at the primary operation. The small number of cases of gastroschisis-associated small-bowel atresia seen in any one unit may conceal the importance of the problem, and limits experience in the approach to management.

The resolution of aneuploid DNA stem lines by flow cytometry: limitations imposed by the coefficient of variation and the percentage of aneuploid nuclei.

Factors important in the resolution of cell sub-populations with differing DNA contents were investigated using an EPICS C flow cytometer. Software is available for the EPICS C which permits data from any two histograms to be superimposed or added together before display. Samples of fresh and archival thyroid tissue, stained with propidium iodide, were analysed on the flow cytometer and the peak channel number noted. The photomultiplier (PMT) voltage was increased and the sample analysed again producing a second histogram with a higher peak channel number. The two histograms were added together to simulate a cell suspension with two sub-populations with a different DNA content. By systematically altering the PMT voltage and the number of nuclei included in each analysis, it was possible to examine the importance of DNA index and the percentage of tumor cells with an aneuploid DNA content for both fresh and paraffin-embedded thyroid nuclei. The crucial importance of achieving a low coefficient of variation (CV) was demonstrated and consequently the reservations that pertain when archival material is studied, particularly in tumours where DNA aneuploidy is frequently expressed with a low DNA index.

Outcome of surgery for Graves' disease re-examined.

The outcome of surgery for Graves' disease in terms of early and late morbidity was studied in 161 patients undergoing subtotal thyroidectomy in the 10-year period 1976-1985. Eighty of these patients had a minimum follow-up of 5 years. There was a low operative morbidity and a zero mortality. The weight of thyroid tissue preserved (in the range 5-10 g) influenced the prevalence of hypothyroidism at one year and at five years. There was a cumulative incidence of hypothyroidism which could not be reliably predicted from biochemical results during the first year. Over 60 per cent of patients with subclinical hypothyroidism at 4 months (63 per cent) or 1 year (70 per cent) did not subsequently need thyroxine replacement within 5 years. Patients remained at risk of developing recurrent toxicity indefinitely and the risk was significantly greater in patients with small goitres (less than 50 g). Our results may be improved by leaving larger remnants (9-10 g) in most patients and smaller remnants (2-4 g) in those with small glands in whom alternative treatment, which is to be preferred, is not acceptable. After subtotal thyroidectomy for Graves' disease lifelong follow-up is necessary.

Prophylactic gastropexy in the asplenia syndrome.

The asplenia [Ivemark] syndrome (AS) is the association of congenital absence of the spleen with a variety of visceral abnormalities, predominantly of the cardiovascular system. Varying degrees of malrotation and malfixation of the bowel are common in this condition, and the occurrence of catastrophic gastric volvulus due to malfixation of the bowel has been reported. With the improvement in long-term outlook for these patients with modern cardiac surgery and prophylactic antibiotics, the intra-abdominal anomalies have become increasingly significant. This paper draws attention to the prophylactic treatment of gastric malfixation in the AS through the presentation of two cases in which gastropexy was performed.