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Aim of the Study: Insertion of a thoraco amniotic shunt (TAS) during fetal life is a therapeutic option where there is a high risk of death secondary to large congenital lung malformations (CLM). The aim of this study is to present our center's long-term experience., Methods: Retrospective single center review of the period (Jan 2000-Dec 2020). We included all fetuses that underwent TAS insertion for CLM with detailed analysis of those live newborns managed in our center. Data are quoted as median (range)., Main Results: Thirty one fetuses underwent 37 TAS insertions at a 25 (20-30) weeks gestational age. This was successful on 1st attempt in 30 (97%) fetuses. In 6 cases a 2nd shunt was required at 6.5 (2-10) weeks following the 1st insertion. Twenty-eight survived to be born. Sixteen (9 male) infants were delivered in our center at 39 (36-41) weeks gestational age and birth weight of 3.1 (2.6-4.2) kg. All infants underwent surgery at 2 (0-535) days (emergency surgery, n = 9; expedited n = 4; elective surgery, n = 3). Final histopathology findings were CPAM Type 1 (n = 14, n.b. associated with mucinous adenocarcinoma, n = 1), CPAM Type 2 (n = 1) and an extralobar sequestration (n = 1). Postoperative stay was 16 (1-70) days with survival in 15/16 (94%). One infant died at 1 day of life secondary to a combination of pulmonary hypoplasia and hypertension. Median follow up period was 10.7 (0.4-20.4) years. Nine (60%) children developed a degree of chest wall deformity though none have required surgical intervention. Clinically, 14/15 (93%) have otherwise normal lung function without limitations of activity, sporting or otherwise. One child has a modest exercise limitation (FVC - 70% predicted)., Conclusions: TAS insertion is associated with high perinatal survival and should be considered in fetuses at risk of hydrops secondary to large cystic lung malformation. Their long term outcome is excellent although most have a mild degree of chest wall deformity., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Aim of the Study: Congenital pulmonary airway malformation (CPAM) has an estimated prevalence in Europe of 1.06/10,000 live births with most being detected using maternal ultrasound screening. Malignant transformation is a possible complication though its prevalence is unknown and previous reports have usually been in older children. We reviewed our experience to identify those CPAM cases associated with malignancy., Methods: Single centre retrospective review of all surgically treated children with antenatally-detected CPAM, with detailed review of cases associated with malignancy., Main Results: 210 infants and children underwent resectional surgery for CPAM during the period 1994-2020, with 43(20.5%) undergoing surgery during the neonatal period. Of these, 3 infants, all males, had undergone surgical resection for respiratory distress (at 3, 4 and 8 days of life) with subsequent histological confirmation as Stocker type 1 CPAM with clear foci of mucinous adenocarcinoma. Subsequent genetic analysis showed somatic KRAS (Kirsten Rat Sarcoma Viral Oncogene) mutations in all three cases. No adjuvant treatment was required, and all are asymptomatic and disease-free at most recent follow-up (8 months, 2 and 6 years) CONCLUSIONS: This series highlights a clear association between type 1 CPAM and mucinous adenocarcinoma with KRAS point mutations, suggesting that the process of carcinogenesis has the potential to start in utero. This underlines the importance of discussing the risk of malignancy in prenatal and postnatal counselling., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Aims: The optimal management for boys under 3 months of age with an indirect inguinal hernia (IIH) and ipsilateral palpable undescended testis (IPUDT) is unknown. We aimed to: 1) determine the current practice for managing these boys across the UK, and 2) compare outcomes of different treatment strategies., Methodology: We undertook two studies. Firstly, we completed a National Survey of all surgeons on the British Association of Paediatric Surgeons email list in 2014. Subsequently, we undertook a multi-centre, retrospective, 10-year (2005-2015) review across 4 pediatric surgery centers of boys under 3months of age with concomitant IIH and IPUDT. Primary outcome was testicular atrophy. Secondary outcomes included need for subsequent orchidopexy, testicular ascent and hernia recurrence. Data are presented as median (range). Chi-squared test and multivariate binomial logistic regression analysis were used for analysis; p<0.05 was considered significant., Results: Survey: Consultant practice varies widely across the UK, with a tendency towards performing concurrent orchidopexy at the time of herniotomy under 3 months of age. Concurrent orchidopexy is favored less in cases where the hernia is symptomatic. Case Series Review: Forty-one boys with 43 concomitant IIH and IPUDT were identified, and all included. 32 (74%) hernias were reducible, 11 (26%) were symptomatic requiring urgent or emergency repair. Post-conceptual age at surgery was 45weeks (36-65). Primary operations included: 29 (67%) open hernia repair and standard orchidopexy, 8 (19%) open hernia repair with future orchidopexy if required, 4 (9%) laparoscopic hernia repair with future orchidopexy if required, 2 (5%) open hernia repair and suturing of the testis to the inverted scrotum without scrotal incision. Variation in atrophy rate between different surgical approaches did not reach statistical significance (p=0.42). Overall atrophy rate was 18%. If hernia repair alone was undertaken (8 open and 4 laparoscopic), the testis did not descend in 8 patients, requiring subsequent orchidopexy (67%); if orchidopexy was undertaken at the time of hernia repair, 1 in 29 required a repeat orchidopexy (3%) (p=0.0001). No hernia recurred., Conclusion: This study suggests that orchidopexy at the time of inguinal herniotomy does not increase the risk of testicular atrophy in boys under 3months of age., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Introduction: Economic disadvantage may adversely influence the outcomes of infants with gastroschisis (GS). Gastroschisis International (GiT) is a network of seven paediatric surgical centres, spanning two continents, evaluating GS treatment and outcomes., Material and Methods: A 2-year retrospective review of GS infants at GiT centres. Primary outcome was mortality. Sites were classified into high, middle and low income country (HIC, MIC, and LIC). MIC and LIC were sometimes combined for analysis (LMIC). Disability adjusted life years (DALYs) were calculated and centres with the highest mortality underwent a needs assessment., Results: Mortality was higher in the LICs and LMICs: 100% in Uganda and Cote d'Ivoire, 75% in Nigeria and 60% in Malawi. 29% and 0% mortality was reported in South Africa and the UK, respectively. Septicaemia was the commonest cause of death. Averted and non-avertable DALYs were nil in Uganda and Cote d'Ivoire (no survivors). In the UK (100% survival) averted DALYs (met need) was highest, representing death and disability prevented by surgical intervention. Performance improvement measures were agreed: a prospectively maintained GS register; clarification of the key team members of a GS team and management pathway., Conclusions: We propose the use of GS as a bellwether condition for assessing institutional capacity to deliver newborn surgical care. Early access to care, efficient multidisciplinary team working, appropriate resuscitation, avoidance of abdominal compartment syndrome, stabilization prior to formal closure and proactive nutritional interventions may reduce GS-associated burden of disease in low resource settings., (Crown Copyright © 2016. Published by Elsevier Inc. All rights reserved.)

Aims of the Study: Laparoscopic simulation has transformed skills acquisition for many procedures. However, realistic nonbiological simulators for complex reconstructive surgery are rare. Life-like tactile feedback is particularly difficult to reproduce. Technological innovations may contribute novel solutions to these shortages. We describe a hybrid model, harnessing 3D technology to simulate laparoscopic choledochal surgery for the first time., Methods: Digital hepatic anatomy images and standard laparoscopic trainer dimensions were employed to create an entry level laparoscopic choledochal surgery model. The information was fed into a 3D systems project 660pro with visijet pxl core powder to create a free standing liver mold. This included a cuboid portal in which to slot disposable hybrid components representing hepatic and pancreatic ducts and choledochal cyst. The mold was used to create soft silicone replicas with T28 resin and T5 fast catalyst. The model was assessed at a national pediatric surgery training day., Results: The 10 delegates that trialed the simulation felt that the tactile likeness was good (5.6/10±1.71, 10=like the real thing), was not too complex (6.2/10±1.35; where 1=too simple, 10=too complicated), and generally very useful (7.36/10±1.57, 10=invaluable). 100% stated that they felt they could reproduce this in their own centers, and 100% would recommend this simulation to colleagues., Conclusion: Though this first phase choledochal cyst excision simulation requires further development, 3D printing provides a useful means of creating specific and detailed simulations for rare and complex operations with huge potential for development., (Copyright © 2016. Published by Elsevier Inc.)

Background: Gastroschisis associated intestinal dysmotility (GAID) is poorly understood. Animal experiments suggest that interstitial cells of Cajal (ICC), play an important role., Methods: Infants with gastroschisis (GS) and GAID (time to full feed >42days) were selected. Age matched GS and control (NEC, ileal atresia, malrotation, and volvulus) samples from primary (T1) and secondary (T2) time points underwent standard histopathology and immunohistochemistry for identification of ICC, followed by evaluation of ICC numbers, distribution, morphology, relation to ganglion cells, and myenteric plexus architecture. Groups were compared using parametric and nonparametric tests., Main Results: Twelve patients had samples available for histopathological evaluation. GAID patients had a significantly lower total number of ICCs than controls (3 vs. 8, P<0.0029). ICC number at T1 was 2.5 vs. 6 (P=0.0629) and significantly lower at T2. (3.5 vs. 11, P=0.0124). GAID patients did not show a significant increase of ICC from T1 to T2. Controls showed a significant increase of ICC over time (6 vs. 11, P=0.0408)., Conclusion: Intestinal samples from infants with GAID who underwent stoma formation demonstrated fewer ICC than controls. There was no improvement or cell recovery during the study period. The ability to modulate ICC may have significant implications for the management of GAID., (Copyright © 2015. Published by Elsevier Inc.)

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., (Copyright © 2014 The Authors. Published by Elsevier Inc. All rights reserved.)

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.

Background: The "clip and drop" (CD) has been proposed as a useful strategy in the management of severe multifocal necrotizing enterocolitis (NEC). There is little published data on clinical outcomes after this intervention. We report a 2-center experience with this technique., Methods: A retrospective review of infants who underwent CD between 1998 and 2006 at 2 tertiary pediatric surgery centers. Data recorded included intestinal resections, interval between laparotomies, anastomoses at subsequent surgery, time to full feeds, and complications including mortality. Data are reported as median with ranges., Results: Thirteen infants (7 male, 6 female) with a birth weight of 811 (514-2110) g underwent CD of up to 5 bowel segments. In 8 of 9 early survivors, all CD segments were viable. Six patients (46%) were alive at 29 (9-96) months. Survivors underwent 4 (3-4) laparotomies and 4 (2-6) bowel anastomoses and had intestinal continuity restored at 67 (51-162) days., Conclusions: With multiple interventions, half the infants in this high-risk group survived and achieved full enteral feeds. The CD is a valuable technique in a selected group of infants with fulminant NEC.

Introduction: A parent is often present during anesthetic induction of their child. Some ask to see surgery. We sought views regarding the prospect of a parent in theater during surgery., Methods: A questionnaire survey of parents, theater staff, and surgeons was used. Visual analog scales were also used. A standard error of the mean was calculated for each parameter. Statistical analysis was by Student's t test. Comparisons were made between groups, and a P value of less than .05 was considered significant., Results: Three hundred seven respondents--204 parents, 75 theater personnel, and 28 surgeons. Parents favored the option to be present in theater. Across groups, support declined with intensity of intervention; minor surgery under local anesthetic, parental score of 8.43, declining to 6.5 for minor elective surgery under general anesthetic, and 5.1 for emergency surgery. There were also declines for theater personnel (2.7, 1.1, and 0.9) and surgeons (4.29, 1.5, and 0.6). Scores for theater personnel and surgeons were significantly lower than the parents (P < .001)., Conclusion: This study confirms a desire by parents to be present in theater during surgery on their child but demonstrates the concerns of professionals. We propose a randomized study to test the hypothesis that having a parent in theater has measurable benefits.

Purpose: Gastroschisis (GS) is defined as a full-thickness abdominal wall defect (usually right-sided) with intestinal prolapse and occasionally other viscera. The defect itself may close around the viscera causing exit or entry level intestinal atresia and ischaemia or midgut infarction, previously described as closed GS. We now report the largest series of infants born with various stages of closing GS describing features, management, and outcome., Methods: The study used a single-centre retrospective review of infants with GS and evidence of defect closure at birth., Results: Nine infants (6 girls) with a median of 35 (range, 32-36) weeks of gestation fulfilled criteria for closing GS from a series of 146 (6%) infants born from August 1994 to December 2007. Delivery had been expedited in 6 based on increasing intraabdominal bowel dilatation and suspicion of closing GS on serial antenatal ultrasound. At delivery, 5 had compromised but viable bowel and required intestinal surgery. Three fetuses, all with midgut necrosis, had antenatal scans typical of GS with no additional features. After a variety of surgical procedures, 7 patients are now fully enterally fed, one is parenteral nutrition-dependent. One died of end-stage liver failure secondary to short bowel syndrome. The length of follow-up was 6 (range, 0.5-11) years., Conclusions: Of infants with GS, 6% present with closing abdominal ring. Close antenatal monitoring may prevent severe bowel loss in some cases. After multiple surgical interventions, most have a favorable outcome.

Purpose: We report our experience of the management of arterial occlusion in the newborn., Methods: A case note review was carried out after ethical approval. Doppler ultrasonography confirmed the occlusion. Thrombolysis was the primary intervention. Surgery was used selectively. A good outcome was one without tissue loss or functional impairment or minimal tissue loss without functional impairment. Data are presented as medians with ranges., Results: Ten patients (9 male; median gestational age, 35.5 weeks [range, 28-39 weeks]) presented on day 1 (range, 1-8 days). Initial management included systemic tissue plasminogen activator (8 patients) and surgery (2 infants in whom thrombolysis was contraindicated). Improvement was noted in 7 of 8 infants treated medically and in both who underwent surgery. Three infants had significant tissue loss. Outcome at 29 months (range, 1.3-95.4 months) was good in the remaining 7., Conclusions: A multidisciplinary approach, thrombolysis and selective surgery achieved tissue preservation and function in the majority while minimizing complications. Early referral to centers with multidisciplinary teams is recommended.

Introduction: Early elective delivery of antenatally diagnosed gastroschisis has been proposed as a strategy to minimize postnatal morbidity. This hypothesis was tested by analyzing outcome in relationship to gestational age and birth weight at delivery., Methods: Single-center retrospective review of infants born with gastroschisis over a 13-year period (January 1993-December 2005). Standard outcome measures were compared using nonparametric methods. Data are quoted as median values (range)., Results: The study population consisted of 110 infants with gastroschisis. They were divided according to gestational age (group A, <35 weeks; group B, 35-37 weeks; group C, >37 weeks) and birth weight (group D, <2 kg; group E, 2-2.5 kg; group F, >2.5 kg). Duration in hospital (P < .01) and time to full enteral feeding (P = .05) was increased in group A vs groups B and C. In comparison, duration in hospital (P < .01), days ventilated (P = .03), establishment of full feeds (P = .01), and parenteral nutrition (P = .02) were all prolonged in group D vs groups E and F. Six (5%) infants died (group D, n = 3; group E, n = 3). Necrotizing enterocolitis was found in 7 infants, and confined to groups D and E (chi2 for trend P = .06)., Conclusion: There is no evidence that prematurity confers an advantage in restitution of gastrointestinal function in infants with gastroschisis; indeed, the opposite appears true. Birth weight, rather than gestational age, appears a better predictor of outcome.

Background: The management of children with main pancreatic duct injuries is controversial. We report a series of patients with pancreatic trauma who were treated using minimally invasive techniques., Methods: Retrospective review of children with pancreatic trauma treated at a UK tertiary referral institution between 1999 and 2004., Results: Fifteen children (11 boys) were admitted with pancreatic trauma. Twelve (80%) were less than 50th centile for body weight. Contrast-enhanced computed tomography (CT) scans were used to define organ injury, supplemented by magnetic resonance cholangiopancreatography (MRCP) in 7. Twelve (80%) underwent diagnostic endoscopic retrograde cholangiopancreatography (ERCP) with a median time after injury of 11 (range, 6-29) days. The degree of pancreatic injury was defined by ERCP and CT/MRCP as grade II (n = 2), grade III (n = 4), grade IV (n = 9) (American Association for the Surgery of Trauma grades). Nine children had a transductal pancreatic stent inserted endoscopically. Computed tomography/ultrasound-guided drainage was performed in 4 children for acute fluid collections. Two children later underwent endoscopic cyst-gastrostomy, one of whom later required conversion to an open cyst-gastrostomy., Conclusion: Body habitus may predispose to pancreatic duct trauma. Contrast-enhanced CT scan (and MRCP) should dictate the need for ERCP. Transductal pancreatic stenting allows internal drainage of peripancreatic collections and may reestablish duct continuity, although a proportion still requires percutaneous or endoscopic cyst-gastrostomy drainage. Open surgery for pancreatic trauma should now be an exception.

Background/purpose: Antenatally detected liver cysts are rare; their diagnostic accuracy is unknown, and their management is controversial. This study assessed the natural history of these lesions., Methods: We conducted a retrospective review of infants with isolated intrahepatic cysts that were detected antenatally. Data are expressed as median (range)., Results: Fifteen infants presented during the period 1991-2004 with an antenatally detected liver cyst. Their gestational age at detection was 22 (18-34) weeks, and the maximum diameter of their cyst was 23 (10-120) mm. Serial scans, which were performed in 9 fetuses, showed cyst enlargement in 5 cases, diminution in 1 case, and no change in 3 cases. In utero percutaneous aspiration was required in 1 infant. Three infants underwent postnatal surgery. One fetus (postfetal intervention) had a subtotal excision of a large subcapsular cyst filling the abdominal cavity on day 2. Another infant required partial excision and marsupialization (complex cyst arising from segment IV) at 5 months, and a third infant underwent a cyst cholecystostomy at 4 months. Postnatal investigations (including hepatic scintigraphy) suggested that the remaining lesions were either simple parenchymal (n = 10) or isolated intrahepatic choledochal (ie, type V; n = 2) cysts. The median follow-up for these patients was 44 (27-167) months. Serial postnatal ultrasonography showed cyst diminution in 4 cases, an enlargement in 1 case, and no dimensional change in 7 cases., Conclusions: Most antenatally detected liver cysts appear to be simple and of parenchymal origin and do not require fetal intervention. Their postnatal history is variable, but regression without treatment is seen in most cases.

Aims: Sacrococcygeal teratomas (SCTs) are the commonest neonatal tumors with an incidence of approximately 1:30,000. There are few large single-center series and even fewer describing both their antenatal and postnatal course. We report the outcome of all fetuses investigated at a tertiary fetal medicine center with this diagnosis., Method: Demographic details were obtained from a prospectively maintained database. Patient records were examined for additional data including antenatal and postnatal interventions. Data were described as median (range)., Results: Forty-one SCTs were diagnosed antenatally during the period 1993 to 2004. Twelve were excluded from subsequent analysis (single antenatal visit or attending for second opinion [n = 6] and termination of pregnancy [n = 6]). Twelve underwent fetal intervention (laser vessel ablation [n = 4], alcohol sclerosis [n = 3], cyst drainage [n = 2], amniodrainage [n = 2], vesicoamniotic shunt [n = 1]) for fetal hydrops and polyhydramnios to aid in delivery and to prevent obstructive uropathy developing in the fetus. Of these, 3 died in utero and 9 survived to be born (median gestational age, 33 weeks [27-37 weeks]). A further 3 died in the neonatal period. There are 6 long-term survivors (50%) from this group. Seventeen infants, without intervention, were born at median gestational age 38 weeks (26-40 weeks). One infant with severe cardiac anomalies died on the day of birth. All surviving infants had definitive excisional surgery at a median of 2 days (1-16 days). Current median follow-up of survivors is 39 months (8-86 months). There have been no recurrences. One child has mild constipation, and 3 are awaiting cosmetic revision of their scars., Conclusions: The overall survival of antenatally diagnosed SCT is approximately 77%, with the development of hydrops and others requiring in utero intervention carrying a poor prognosis. Otherwise, the outcome after surgical excision is excellent.