Shailesh Patel

Best practice guidelines: Fetal surgery

Fetal intervention encompasses a range of procedures on the fetus with congenital structural anomalies, whilst still on the placental circulation. The concept of fetal surgery was conceived in order to prevent fetal or early postnatal death, or to prevent permanent irreversible organ damage. The benefit of these procedures has to be balanced with risks to both the mother and the fetus. Open fetal surgery, more commonly conducted in North American centres, involves open surgery to the uterus in order to operate on the fetus. Fetal intervention centres in Europe more commonly use minimally invasive fetoscopic surgery. This paper elaborates on the various strategies used in dealing with anomalies of different organ systems of the fetus.

Systematic review and meta-analysis of the postnatal management of congenital cystic lung lesions

BACKGROUND Antenatally detected asymptomatic congenital cystic lung lesions may be managed conservatively or by surgical resection. We undertook a systematic review and meta-analysis to quantify the risks of elective surgery, emergency surgery, and observation. METHODS All series published between 1996 and 2008, where the postnatal management of congenital cystic lung lesions was described, were reviewed. A meta-analysis was performed to determine whether elective or emergency surgery was associated with a higher risk of adverse outcomes. RESULTS There were 41 reports describing 1070 patients (of whom 79% were antenatally detected). Five hundred five neonates survived without surgery into infancy, of whom only 16 (3.2%) became symptomatic. For all ages, elective surgery was associated with significantly less complications than emergency surgery. The risk ratio was 2.8 (95% confidence interval, 1.4-5.5; P < .005) when comparing complications after elective surgery with emergency surgery. CONCLUSIONS The risk of asymptomatic cases developing symptoms is small. However, elective surgery is associated with a better outcome than emergency surgery. If elective surgery is undertaken, it should be performed before 10 months. Although no prognostic indicators have so far been identified in the literature, a conservative approach may be appropriate for small lesions.

Is early delivery beneficial in gastroschisis

PURPOSE Gastroschisis neonates have delayed time to full enteral feeds (ENT), possibly due to bowel exposure to amniotic fluid. We investigated whether delivery at <37weeks improves neonatal outcomes of gastroschisis and impact of intra/extra-abdominal bowel dilatation (IABD/EABD). METHODS A retrospective review of gastroschisis (1992-2012) linked fetal/neonatal data at 2 tertiary referral centers was performed. Primary outcomes were ENT and length of hospital stay (LOS). Data (median [range]) were analyzed using parametric/non-parametric tests, positive/negative predictive values, and regression analysis. RESULTS Two hundred forty-six patients were included. Thirty-two were complex (atresia/necrosis/perforation/stenosis). ENT (p<0.0001) and LOS (p<0.0001) were reduced with increasing gestational age. IABD persisted to last scan in 92 patients, 68 (74%) simple (intact/uncompromised bowel), 24 (26%) complex. IABD or EABD diameter in complex patients was not significantly greater than simple gastroschisis. Combined IABD/EABD was present in 22 patients (14 simple, 8 complex). When present at <30weeks, the positive predictive value for complex gastroschisis was 75%. Two patients with necrosis and one atresia had IABD and collapsed extra-abdominal bowel from <30weeks. CONCLUSION Early delivery is associated with prolonged ENT/LOS, suggesting elective delivery at <37weeks is not beneficial. Combined IABD/EABD or IABD/collapsed extra-abdominal bowel is suggestive of complex gastroschisis.

Nipple pain at presentation predicts success of tongue-tie division for breastfeeding problems.

INTRODUCTION In mother-infant pairs experiencing breastfeeding difficulties, frenulotomy for tongue-tie may improve breastfeeding. We tested the hypothesis that those experiencing nipple pain are most likely to benefit from the procedure in a prospective cohort study. MATERIALS AND METHODS Mother-infant pairs attending a dedicated clinic for the assessment and treatment of tongue-tie completed a standardised, structured symptom questionnaire. Three months later outcome was assessed by questionnaire. Multivariate logistic regression analysis was used to determine preoperative predictors of successful outcome. RESULTS Sixty-two infants <90 days old underwent frenulotomy and completed follow-up. At presentation, 52 mothers (84%) reported nipple pain, and 32 mothers (52%) nipple trauma. Three months after frenulotomy, 78% of respondents were still breastfeeding. Feed lengths (mean reduction: 17 mins; p<0.001) and time between feeds (mean increase: 38 mins; p<0.001) had significantly improved, as had difficulty of feeding (mean improvement in self-rated difficulty score: 42%; p<0.001). Those having difficulty breastfeeding due to nipple pain showed a significant long-term benefit from frenulotomy; pre-frenulotomy nipple pain was associated with an increased likelihood of breastfeeding at 3 months in adjusted multivariate analysis (OR 5.8 [95% CI 1.1-31.6]). CONCLUSION Mother-infant pairs with tongue-tie and breastfeeding difficulties due to nipple pain are most likely to benefit from frenulotomy.

Absence of the ductus venosus.

Absence of the ductus venosus is a rare vascular anomaly, but clinicians should be aware that it can be diagnosed antenatally and the prognosis is dependent on the type of associated malformation of the fetal vascular system. Antenatal detection of a single umbilical artery and unexplained cardiomegaly should prompt detailed examination of the umbilical and portal veins. Absent ductus venosus is associated with three main patterns of abnormal venous circulation, the worst prognosis being seen when the umbilical vein bypasses the liver and connects to the right atrium. Fetuses with absence of the ductus venosus are at risk of other congenital anomalies including facial clefts, hemivertebrae, cardiac, genitourinary, gastrointestinal anomalies; affected infants also have a poorer prognosis. In conclusion, fetuses with features suggestive of absence of ductus venosus require referral to a tertiary perinatal center.

Portacaths are safe for long-term regular blood transfusion in children with sickle cell anaemia

Peripheral venous access in children with sickle cell anaemia (SCA) requiring regular blood transfusions can become difficult over time. Previous reports have suggested the use of totally implantable venous access devices, Portacaths (PAC) in this patient group are associated with unacceptable high rates of complications. We present our experience in seven children with SCA over a 9-year period. Seven devices were placed for a total of 9754 PAC days during the study period. The median age at insertion was 6.3 years (range 3–15 years). The rate of PAC associated infection was 0.2 per 1000 PAC days. There were no episodes of thrombosis. The median length of time in situ during the study period was 3.7 years (range 1.3–7.5 years). Our experience highlights the safe and reliable use of PAC in children with SCA requiring regular blood transfusions when venous access has become a major problem.

Why do general dental practitioners refer to a specific specialist endodontist in practice

AIMS To identify the factors that influence the decision of general dental practitioners (GDPs) in Northern Ireland to refer to a specific specialist endodontist. METHODOLOGY A self-administered questionnaire was sent to 220 GDPs in Northern Ireland. The questionnaire comprised questions on demographic characteristics, pattern of practice, pattern of referral and factors influencing the decision to refer to a specific specialist endodontist in practice. The data were analysed using descriptive statistics and the chi-squared (χ(2) ) test at the 0.05 level of significance. RESULTS The response rate was 81%. All respondents stated that they carried out root canal treatment, and the majority (83%) stated that they also carried out root canal retreatment. A minority of respondents (11%) stated that they carried out surgical endodontics. These individuals were more likely to be men, hold a postgraduate qualification, or work in a rural location. The majority of respondents (94%) referred patients with an endodontic problem. These individuals were more likely to be women, not hold a postgraduate qualification, or not carry out surgical endodontics. GDPs indicated a preference for referring to a specialist endodontist in practice over other treatment providers. Factors considered to be of importance in the decision to refer to a specialist endodontist in practice included the practice location of, reputation of, communication with and patient management by the specialist endodontist. The greatest proportion of respondents ranked short waiting time for a consultation as the top promoter when referring to a specific specialist endodontist in practice. CONCLUSION The decision by GDPs to refer to a specific specialist endodontist in practice is multifactorial and influenced by several factors independent from the nature of endodontic disease. In Northern Ireland, the top promoter for referring to a specific specialist endodontist in practice was a relatively short waiting time for a consultation.

Preformed Silos versus Traditional Abdominal Wall Closure in Gastroschisis: 163 Infants at a Single Institution

INTRODUCTION The surgical management of gastroschisis (GS) is controversial. The most commonly used strategy for abdominal wall closure is surgery on day 1 of life with the aim of primary closure (PC) or construction of a surgical silo (SS) and secondary closure thereafter. The other widely used technique is application of a preformed silo (PFS) and reduction of contents over a few days before final closure. There is still a paucity of comparative outcome data. METHODS A retrospective case note review of all infants initially treated at a single institution between October 1993 and October 2012. PFS was adopted as the technique of choice in April 2005. Infants with closed or closing GS were excluded. Data are presented as median (range). p < 0.05 were significant. RESULTS There were 163 infants (156 complete data sets). PFSs were applied in 67 infants and PC/SS were applied in 89 infants of whom 19 infants required a SS. There was no statistical difference between gestational age (p = 0.8), birth weight (p = 0.7), time to first (p = 0.07) and full enteral feeding (p = 0.08), length of hospital stay (p = 0.17), or necrotizing enterocolitis (p = 0.4) and mortality (p = 0.4). Infants treated with PC + SS were closed on day 0 (range, 0-11 days) versus day 6 (range, 2-22 days) of life (p < 0.001). PC + SS were ventilated for day 5 (range, 1-22 days) versus day 3.5 (range, 0-20 days) days (p = 0.01). CONCLUSION Infants treated with PFS required less ventilation than those treated by PC + SS. There was no difference in time to full feeds, length of hospital stay mortality or morbidity.

Pancreatic Pseudocysts in Children: Treatment by Endoscopic Cyst Gastrostomy

Aim: The aim of the present study was to review the use of endoscopic cyst gastrostomy (E-CG) as a treatment option for pancreatic pseudocysts referred to a tertiary paediatric surgical centre. Methods: Retrospective review during a 10-year period (January 2001–December 2010). Cyst gastrostomies were performed using 1 or 2 double pigtailed Zimmon stents (7–10 Fr) under general anaesthesia. Data are quoted as median (range). Results: E-CG was performed in 7 (5 males) children (median age at presentation 11.7 [8.2–15.8] years). Pancreatic pseudocysts were caused by acute pancreatitis in 5 (gallstones n = 1, hereditary pancreatitis n = 1, pancreatic divisum n = 1, asparaginase induced n = 1, and idiopathic n = 1) and pancreatic trauma in 2 (motor vehicle accident n = 1, and handlebar injury n = 1). All of the cases were associated with a rise in serum amylase level, median 1028 (276–2077) IU/L at the peak of symptoms. Three children had pancreatic duct stent placement during endoscopic retrograde cholangiopancreatography as the initial therapeutic intervention, but went on to have E-CG later. One who had a huge pseudocyst at presentation had already undergone an open cyst gastrostomy, which had recurred at 1 month. Rescue E-CG was performed 38 days later. All of the stents were removed endoscopically at 8 (6–40) weeks. E-CG was uncomplicated and pseudocysts resolved completely in 5. One required repeat placement at 15 days due to catheter slippage with later full resolution. One child required open cyst gastrostomy due to reaccumulation two months following removal of the stent. Median hospital stay post E-CG was 3 (1–23) days. There has been no recurrence at median follow-up of 18 (5–108) months. Conclusions: Endoscopic cyst gastrostomy is a safe and effective alternative for the management of pancreatic pseudocysts in children and should now be considered as treatment of choice.

Gastroschisis closure: a technique for improved cosmetic repair

Gastroschisis closure is performed either primarily or after staged reduction of the prolapsed bowel. A technique for surgical closure of the abdominal wall defect is described which allows for an almost scarless appearance of the abdominal surface with preservation of a midline umbilicus.