Long-term clinical and socioeconomic outcomes of children with biliary atresia.

Background: Biliary atresia (BA) is rare liver disease of unknown etiology, and is a major indication for liver transplant (LT). Previous data indicate improved outcomes with early referral for Kasai portoenterostomy (KPE). Objective: Evaluate the long-term outcomes in BA, with particular focus on those transitioned to adult care with native livers. Subjects and Methods: Patients with BA treated between1980 and 2012 were identified. Data were collected from the time of referral, transition to adult care, and the most recent clinic notes, from which patient and native liver survival were calculated. Results: Four hundred and fifty-four patients with BA were identified, who were followed up for median of 16.4 years from birth; 74 died (41 of whom had a LT), giving a 20-year survival rate of 83.6%. Two hundred and seventy-two patients received an LT, with the median native liver survival being 35 months. Of patients who transitioned to adult care, 54 of 180 (30.0%) retained their native liver. Of these, 72% (39 of 54) had evidence of chronic liver disease at transition, of whom 8 were subsequently lost to follow-up, 9 were transplanted, and 22 remained stable with compensated liver disease. Of the 15 of 54 patients (28%) with no evidence of chronic disease in their native liver disease at transition, 3 were subsequently lost to follow-up; none received transplants, although 3 patients developed new-onset liver disease. All patients transitioned to adult care completed secondary school education (N = 180), with 49% having attended college/university and 87% being in employment or education at the last follow-up. Of female patients, 34% had at least one pregnancy (27 children in 21 women), while 22% of males had fathered a child. Conclusion: Long-term outcomes in BA are good, with patients surviving into adult life. Progression of chronic liver disease and associated morbidity is common in those who retained their native livers, suggesting that these patients require monitoring of liver disease throughout adult life, and early recognition of the need for LT.

Management of lymphadenitis due to non-tuberculous mycobacterial infection in children.

PURPOSE: Non-tuberculous mycobacterial (NTM) infection is an important cause of cervico-facial lymph node enlargement in young children. The optimal treatment is thought to be early complete excision without chemotherapy. We compared management of patients referred to our centre to this "gold standard" and determined clinical outcomes by type of primary surgical intervention (complete excision vs. incomplete excision). METHODS: Retrospective study of management and clinical outcomes of all children (<12 years) with NTM lymphadenitis referred to a single UK centre between May 1998 and May 2008. RESULTS: We identified 43 children. Median time from onset of swelling to operation was 6 weeks. Management was: no operation (n = 1, 2 %), complete excision (n = 20, 47 %), incision and drainage (n = 17, 40 %) and fine needle aspirate (n = 5, 12 %). Children not treated by primary complete excision were more likely to have: re-operation (91 vs. 30 %; χ(2) = 16.48; p < 0.0001); persistent lymphadenitis (77 vs. 30 %; χ(2) = 9.45; p = 0.002); sinus formation (26 vs. 5 %; χ2 = 3.74; p = 0.05). CONCLUSION: Failure to undertake primary complete excision leads to further morbidity. A high index of suspicion is required for timely appropriate management to avoid unnecessary morbidity and further intervention.

Antenatal bowel dilatation in gastroschisis: a bad sign?

AIM: Foetal bowel dilatation in gastroschisis is traditionally taken to be an indicator of poor prognosis and parents are counselled accordingly. Increased bowel distension is often a factor in the decision for early delivery. The aim of this study was to establish whether a correlation exists between antenatally detected bowel dilatation and the postnatal outcome for babies with gastroschisis. METHODS: Antenatal ultrasound scans and subsequent postnatal notes were reviewed for cases of isolated gastroschisis from 2004 to 2008. Bowel dilatation was defined as >10 mm diameter. The type (intra- and/or extra-abdominal dilatation), maximum bowel diameter and gestation at which bowel dilatation was first seen was recorded. Outcome measures included number of days of TPN, type of closure (primary or delayed) and postnatal complications. RESULTS: There were 60 antenatal cases of gastroschisis over 5 years. Postnatal notes of 47 were obtained. 38 (81%) had antenatal bowel dilatation, 9 did not. Of those with bowel dilatation, 24 underwent primary closure, 9 required silos, 2 a patch and 3 stomas. Seven (15%) had bowel atresia or necrosis. Three died: NEC, septicaemia and CMV hepatitis. The average time on TPN for those without major complications was 22 days. Of those with no bowel dilatation, 4 had primary closure, 3 a silo, 1 a patch and 1 a stoma. Two had complications requiring further surgery, including one atresia resulting in short gut syndrome. There were no deaths. The mean number of days on TPN was 23. The results also demonstrated no significant correlation between gestation when dilatation was first seen, or degree of dilatation, or both these factors combined, and time of TPN requirement. CONCLUSION: These results do not support the current practice of using antenatal bowel dilatation as a prognostic indicator.

Staged reduction of gastroschisis using preformed silos: practicalities and problems.

PURPOSE: Previous single-center studies have reported favorable outcomes when preformed silos (PFS) are used for the staged reduction of gastroschisis. The aim of this study was to assess the frequency and nature of complications associated with PFS in a large population and provide an insight into the practicalities of their routine use. METHODS: A retrospective review was carried out of all cases of gastroschisis managed with PFS in 4 UK neonatal surgical units for a 6-year period. RESULTS: One hundred fifty infants were included, and 139 (92.7%) silos were applied at cot side (no sedation, n = 93). Median silo size was 4 cm, and time of application was 2.5 hours. Enlarging the defect by incision of fascia was required in 17 (11%). Defect closure was performed at a median of 4 days (0-47) with 93 (62%) being at cot side. Methods of closure were adhesive strips/dressings (n = 94), sutures (n = 48), and patch (n = 8). Discoloration of the viscera occurred in 16 (11%), managed successfully by simple methods (change of PFS, aspirating the stomach, or incision of the defect fascia) (n = 8), conversion to operative silo (n = 3), and operative reduction (n = 1). Four required bowel resection. Other complications included missed atresia (n = 5; 3.3%) and nectrotizing enterocolitis (n = 11; 7%). There were 5 deaths in the series (3.3%). CONCLUSIONS: Staged reduction of gastroschisis with PFS is simple, convenient, and safe. The low rates of associated complications and mortality appear favorable when compared to infants managed with more traditional techniques. We recommend that PFS should be used for the routine management of gastroschisis.

Pre-sternal mass with a congenital sternal defect: a rare presentation.

Anterior chest wall and anterior mediastinal infections are very rare in children with no predisposing thoracic surgery. Congenital sternal defects occur as a result of incomplete ossification of the sternal bone, and show a wide range of severity. We present the case of a 15-month-old boy who presented to the A&E department with an anterior chest-wall mass. After investigation, it was found to be an infective mass, with extension into the anterior mediastinum, through a previously undiagnosed 5 mm congenital sternal defect identified peri-operatively. After antibiotic therapy and surgical management the patient made complete recovery with no recurrence or complications within 6 months of review.

An unusual variant of choledochal cyst: a case report.

INTRODUCTION: Choledochal cyst is an uncommon congenital disease of the biliary tract in the UK. There are five main types of choledochal cyst with several recognised sub-types. However, occasional variants do occur. CASE PRESENTATION: We report a case of a female infant with an antenatally diagnosed choledochal cyst. The operative cholangiogram revealed an unusual intrahepatic biliary tree. The cyst was successfully excised and the infant is well at 18-months follow up. CONCLUSION: The anatomy should be clearly defined before surgical excision as abnormal variants can occur, which usually do not fit into the known classification types and subtypes.