Esophageal replacement in children: Challenges and long-term outcomes.

Replacement of a nonexistent or damaged esophagus continues to pose a significant challenge to pediatric surgeons. Various esophageal replacement grafts and techniques have not produced consistently good outcomes to emulate normal esophagus. Therefore, many techniques are still being practiced and recommended with no clear consensus. We present a concise literature review of the currently used techniques and with discussions on the advantages and anticipated morbidity. There are no randomized controlled pediatric studies to compare different types of esophageal replacements. Management and graft choice are based on geographical and personal predilections rather than on any discernible objective data. The biggest series with long-term outcome are reported for gastric transposition and colonic replacement. Comparison of different studies shows no significant difference in early (graft necrosis and anastomotic leaks) or late complications (strictures, poor feeding, gastro-esophageal reflux, tortuosity of the graft, and Barrett's esophagus). The biggest series seem to have lower complications than small series reflecting the decennials experience in their respective centers. Long-term follow-up is recommended following esophageal replacement for the development of late strictures, excessive tortuosity, and Barrett's changes within the graft. Once child overcomes initial morbidity and establishes oral feeding, long-term consequences and complications of pediatric esophageal replacement should be monitored and managed in adult life.

Management of large primary spontaneous pneumothorax in children: radiological guidance, surgical intervention and proposed guideline.

PURPOSE: Primary spontaneous pneumothorax (PSP) is managed in accordance with the adult British Thoracic Society (BTS) guidelines due to lack of paediatric evidence and consensus. We aim to highlight the differences and provide a best practice surgical management strategy for PSP based on experience of two major paediatric surgical centres. METHODS: Retrospective review of PSP management and outcomes from two UK Tertiary Paediatric hospitals between 2004 and 2015. RESULTS: Fifty children with 55 PSP (5 bilateral) were referred to our Thoracic Surgical Services after initial management: 53% of the needle aspirations failed. Nine children (20%) were associated with visible bullae on the initial chest X-ray. Forty-nine children were assessed with computed tomography scan (CT). Apical emphysematous-like changes (ELC) were identified in 37 children (75%). Ten children had also bullae in the asymptomatic contralateral lungs (20%). In two children (4%), CT demonstrated other lung lesions: a tumour of the left main bronchus in one child; a multi-cystic lesion of the right middle lobe in keeping with a congenital lung malformation in another child. Contralateral asymptomatic ELC were detected in 20% of the children: of those 40% developed pneumothorax within 6 months. Best surgical management was thoracoscopic staple bullectomy and pleurectomy with 11% risk of recurrence. Histology confirmed ELC in 100% of the apical lung wedge resections even in those apexes apparently normal at the time of thoracoscopy. CONCLUSION: Our experience suggests that adult BTS guidelines are not applicable to children with large PSP. Needle aspiration is ineffective. We advocate early referral to a Paediatric Thoracic Service. We suggest early chest CT scan to identify ELC, for counselling regarding contralateral asymptomatic ELC and to rule out secondary pathological conditions causing pneumothorax. In rare instance if bulla is visible on presenting chest X-ray, thoracoscopy could be offered as primary option.

Thoracoscopic debridement for empyema thoracis.

BACKGROUND/PURPOSE: The success rate of early thoracoscopic debridement (TD) for childhood empyema was reviewed in light of the increasing reported incidence of empyema associated with pulmonary necrosis (PN). METHODS: Data were collected from 106 patients who underwent thoracoscopic intervention from 2010 to 2016. Twenty additional patients with severe PN/Bronchopleural Fistula (BPF) were not suitable for TD requiring thoracotomy and Serratus anterior digitation flap. RESULTS: 106 patients with a median age of 4 years (IQR 2-6 years) were considered for TD as primary intervention of which 3 needed conversion to thoracotomy. TD alone was successful in 93/106 however, 10 patients required subsequent minithoracotomy for PN/BPF (managed with Serratus anterior digitation flap). Counting conversions as failure, the overall success rate of TD was 88%. No statistical difference was demonstrable in success rate compared to our previous series (93% (106/114) vs 88% (93/106)). CONCLUSIONS: Primary TD in pediatric empyema is associated with an excellent outcome achieving adequate drainage and full expansion of the lung. The majority of failures in our series were attributable to PN/BPF, requiring thoracotomy and Serratus anterior digitation flap. This is likely a consequence of the increasing incidence of necrotizing pneumonia. LEVEL OF EVIDENCE: Level IV.

Congenital lung lesions: Postnatal management and outcome.

Antenatal diagnosis of lung lesion has become more accurate resulting in dilemma and controversies of its antenatal and postnatal management. Majority of antenatally diagnosed congenital lung lesions are asymptomatic in the neonatal age group. Large lung lesions cause respiratory compromise and inevitably require urgent investigations and surgery. The congenital lung lesion presenting with hydrops requires careful postnatal management of lung hypoplasia and persistent pulmonary hypertension. Preoperative stabilization with gentle ventilation with permissive hypercapnia and delayed surgery similar to congenital diaphragmatic hernia management has been shown to result in good outcome. The diagnostic investigations and surgical management of the asymptomatic lung lesions remain controversial. Postnatal management and outcome of congenital cystic lung lesions are discussed.

Customized silicone implant for the correction of acquired and congenital chest wall deformities: A valuable option with pectus excavatum.

BACKGROUND: Surgical remodeling and correction of congenital and acquired chest wall deformities (CWD) is undertaken many times for cosmesis. Although reportedly minimally invasive, commonly used Nuss procedure for correction of pectus excavatum (PE) is not without complications. Nuss procedure is also not suitable for complex deformities and Poland syndrome cases. Insertion of custom-made silicone implants for the reconstruction of defects has been adopted from adult plastic reconstructive surgery as primary repair of CWD or rescue procedure for recurrence of PE after recurrence or residual deformity. METHODS: We present our experience with CWD reconstruction in children with customized silicone prosthesis made from a surgically implantable liquid silicone rubber (NuSil MED-4805, Ca). RESULTS: Since 2006 we treated 26 patients with CWD: six were children (age <17years, median 14.6) with PE. Implants were custom made for each patient's chest. The implants were inserted under general anesthesia. Postoperatively all patients were fully satisfied with the cosmetic result and subjective patient satisfaction remained excellent at follow-up in all the children. Only one child developed postoperative complications (seroma). CONCLUSIONS: Customized silicone implant for PE in the pediatric age is an alternative therapeutic method, as primary or rescue treatment, with equally good cosmetic outcome, fewer significant complications, less postoperative pain and a faster recovery.

A novel technique of permanent self-catheterizable feeding jejunostomy.

Long-term/permanent jejunal feeding in children and adults is prone to significant complications, such as leakage, skin excoriation, and difficulty in reinsertion after dislodgement of the jejunostomy tube. Our innovative jejunostomy technique uses a pedicle segment of jejunum, which is retubularized using the principle of Monti. The jejunal segment is reinserted through a submucosal tunnel back into the jejunum. The other end is brought out onto the abdominal wall. The technique achieves a continent catheterizable jejunal stoma for enteral feeding.

Surgical management of bronchopleural fistula in pediatric empyema and necrotizing pneumonia: efficacy of the serratus anterior muscle digitation flap.

PURPOSE: Surgical management of bronchopleural (B-P) fistula associated with thoracic empyema and necrotizing pneumonia is challenging. We evaluated the treatment and outcome of early surgical intervention with limited decortication and insertion of a serratus anterior muscle digitation flap. METHODS: A retrospective review during a 10-year period of children with empyema and B-P fistula secondary to necrotizing pneumonia was performed. During this period, 335 children with empyema were admitted, of which 20 developed a B-P fistula. All underwent a thoracotomy with insertion of a serratus anterior muscle digitation flap. RESULTS: Prethoracotomy chest computed tomographic scan was performed to define the pathologic characteristic. Streptococcus pneumoniae was identified by pus culture (n = 6) and by polymerase chain reaction (n = 4). The median postoperative time with pyrexia after surgical intervention was 2 days (range, 1-3 days). The median period of thoracostomy drain was 7 days (range, 5-15 days). Length of hospital stay was 21 days (range, 7-43 days). This parenchyma-preserving technique was able to control the B-P fistula successfully in all cases. No reintervention was necessary in any case. Respiratory symptoms and radiology improved in all children on follow-up, except for 3 who required oral antibiotics for subsequent chest infections. CONCLUSION: Early surgical intervention with insertion of a serratus anterior muscle digitation flap is effective and safe and avoids morbidity associated with conservative management and necrotic lung resection surgery.

Management of gastroesophageal reflux associated with malrotation in children.

AIM: Children being investigated for gastroesophageal reflux (GOR) have a high incidence of malrotation. Current literature suggests these patients should be managed with a combined antireflux and Ladd's procedure. We review our experience, the largest series to date, of performing an elective Ladd's procedure as the first-line intervention. METHOD: Retrospective case note review of 20 children with significant symptoms of GOR and an incidental finding of malrotation. Children presenting immediately with bilious vomiting were excluded. All patients underwent a Ladd's procedure as their sole primary operative intervention. RESULTS: Median age at operation was 7 months (21 days-12 years). Fifteen patients (75%) had evidence of reflux on barium contrast study. All children were followed up for at least 6 months. Eighteen (90%) had resolution or significant improvement of their symptoms postsurgery. Only 3 have not managed to tolerate a full oral diet, all unrelated to GOR. None of our series required an antireflux procedure. CONCLUSION: In children with debilitating vomiting necessitating surgical management, a contrast study is imperative in the work up. The high incidence of GOR and the significant improvement after correction of malrotation show the relationship between delayed gastric emptying and GOR. We suggest that when an abnormally placed duodenojejunal flexure is found, a Ladd's procedure alone is sufficient and may obviate the need for a more invasive antireflux procedure.

Mediastinal and pulmonary infantile myofibromatosis: an unusual surgical presentation.

A 4-week-old boy was extensively investigated for stridor and respiratory distress and was found to have a soft tissue mass superior to the left hilum and emphysema of the entire left lung. An exploratory thoracotomy was undertaken for diagnosis and possibly to improve respiratory distress. Intraoperatively, a firm plaquelike mass was identified encasing the entire hilum including left pulmonary artery and left main bronchus. It became apparent that a left pneumonectomy was needed to be performed to resect the tumor completely and achieve hemostasis. Histopathologic examination revealed infantile myofibromatosis with multiple foci within the entire lung parenchyma as well as in the hilar mass. The child is completely recurrence-free and symptom-free after 6 years of follow-up. The literature review was carried out to discuss management of this rare but benign and surgically challenging condition.

Evolving experience with video-assisted thoracic surgery in congenital cystic lung lesions in a British pediatric center.

BACKGROUND/PURPOSE: Video-assisted thoracic surgery (VATS) is increasingly used for the resection of congenital cystic lung lesions (CLLs). This study aimed to evaluate the efficacy of VATS and its outcome in both antenatally and postnatally detected CLLs. METHODS: Forty-six patients managed during 2000-2005 were studied. Demographics, investigations, operative details, and outcome data were collected and evaluated. Patients were divided into 3 groups for analysis. RESULTS: Antenatally diagnosed (groups I and II, n = 35): group I (20) had VATS at 20 months median (range, 16-35 months). Video-assisted thoracic surgery was successful in 14 of 20 (70%), notably in all cases of extralobar sequestrations and foregut duplication cysts. Inadequate vision/lung collapse and technical difficulties were the main reasons for conversion to open thoracotomy. Group II (n = 15) was considered unsuitable for VATS because of neonatal symptoms (6 congenital cystic adenomatoid malformations of the lung [CCAMs]) and/or large size/inexperience (5 CCAMs, 4 sequestrations) and had elective thoracotomy at 8 months median (range, 6 days-20 months). Postnatally diagnosed (group III, n = 11): 3 CCAMs, 6 duplications, and 2 sequestrations were diagnosed because of recurrent chest infection (8) or stridor (2), or incidentally (1) at 8 years median (range, 1.2-14 years). Video-assisted thoracic surgery was successful in 3 foregut duplications. A duplication and an intralobar sequestration were converted; open thoracotomy was performed in others because of previous recurrent pneumonic episodes. Postoperative pain and hospital stay were significantly less (P < .001) in successful VATS resection: median of 2 days (range, 1-7 days) compared with thoracotomy median of 6 days (range, 4-20 days). CONCLUSIONS: Video-assisted thoracic surgery is a safe and effective option for asymptomatic congenital CLLs. It is anticipated that more successful CCAM resections using VATS will occur in the future as our technical ability improves.

Does gastroschisis reduction require general anesthesia? A comparative analysis.

BACKGROUND: Outcome after gastroschisis repair without general anesthesia is controversial, and published conclusions are variable with no comparative studies. AIM: The aim of this study was to present a comparative study evaluating outcome after gastroschisis repair with and without general anesthesia. METHODS: An ambispective nonrandomized study of a cohort of 51 neonates born with gastroschisis between July 1998 and December 2003 was performed. Twenty-four neonates (group 1) had conventional reduction under general anesthesia, and 27 (group 2) cotside minimal intervention reductions were without general anesthesia. RESULTS: Groups were comparable regarding gestational age, birth weight, and quality of eviscerated bowel. Statistical significance (P < .05) was seen between groups 1 and 2 with regard to age at reduction of gastroschisis (5.6 +/- 2.5 vs 3 +/- 1 hours) and time taken for completion of gastroschisis reduction (58.1 +/- 15 vs 49 +/- 14 minutes). No statistical significance (P > .05) was seen with respect to start of feeds (10.4 +/- 3.6 vs 10.9 +/- 4.1 days), duration of total parenteral nutrition (21.5 +/- 7.3 vs 22.4 +/- 6.8 days), and total hospital (stay 29 +/- 10 vs 30 +/- 13 days). Admission to the intensive care unit was required in 92% in group 1 for 1 to 6 days vs 7% in group 2 for 3 to 6 days. There was 1 death in group 1 (4%). Total hospital cost in group 1 was 12,283 pounds sterling +/- 2438 pounds sterling vs 6208 pounds sterling +/- 2120 pounds sterling in group 2 (P = .013). CONCLUSIONS: Neonates with gastroschisis, whose bowel was reduced without general anesthesia, have similar outcomes to those whose bowel was reduced under general anesthesia. Both approaches appear to be safe and effective, but reduction without general anesthesia was cost-effective.

An unusual late presentation of a congenital diaphragmatic hernia.

An unusual and late-presenting case of left Bochdalek diaphragmatic hernia is presented to highlight the fact that the timing and mechanism of presentation of the hernia remains obscure. In this case there were two previous admissions, initially for a left upper lobe lobectomy for congenital lobar emphysema and a subsequent admission for bronchiolitis. He then presented at the age of nine months, with acute respiratory distress and was diagnosed to have a left diaphragmatic hernia. This is the first case of a late- presenting congenital diaphragmatic hernia reported in association with a previous ipsilateral lobectomy for congenital lobar emphysema. A brief review of late -presenting congenital diaphragmatic hernias is given and an alternative theory is proposed for the mechanism of late presentation.