Foregut duplications in the superior mediastinum: beware of a common wall with the tracheo-bronchial tree.

BACKGROUND: Foregut duplication cysts (FD) with a common muscular wall with the oesophagus are well recognised. Our case series highlights the existence of a common wall between superior mediastinal FD and the tracheo-bronchial tree. MATERIALS AND METHODS: Over the last 15 years, we have thoracoscopically resected 41 FD. Five cases were identified to have a common wall with the tracheo-bronchial tree at operation. The clinical history, radiology, findings at operation and pathology were evaluated to highlight learning points. RESULTS: Five superior mediastinal cysts with a common wall were identified, with two antenatally and three postnatally diagnosed. All three postnatal cases and one antenatal case presented with symptoms of respiratory compromise and stridor. Only one neonate was relatively asymptomatic before resection. CT similarities in all cases were: separation of trachea and oesophagus by the cyst, oesophageal deviation to the right or compression and compression of trachea/bronchus. Thoracoscopically, two cysts were resected without injury to the airway, while three had inadvertent tracheo-bronchial injury. Repair of the tracheal injury was possible in one case thoracoscopically, while two cases required conversions, as adequate oxygenation could not be maintained despite on-table endotracheal tube advancement beyond the injury and thoracoscopic manoeuvres. All cases had excellent outcomes at follow-up (median 25months, range 4-39months) with resolution of symptoms and no recurrences. CONCLUSIONS: Our report highlights the existence of a common wall between the superior mediastinal FD and the tracheo-bronchial tree. Thoracoscopic resections are feasible including repair of inadvertent airway injury, provided adequate oxygenation can be maintained.

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.

Use of a novel laparoscopic gastrostomy technique in children with severe epidermolysis bullosa.

OBJECTIVE: Supplementing nutrition in children with severe epidermolysis bullosa (EB) is challenging because of skin and mucosal fragility. Percutaneous endoscopic gastrostomy is contraindicated in EB, whereas more invasive open surgical gastrostomy placement can be complicated by chronic leakage. The aim of the study was to review the efficacy and acceptability, in children with severe EB, of our modified 2-port laparoscopic approach using the Seldinger technique with serial dilatation and tube insertion through a peel-away sheath. METHODS: Retrospective review of children with EB who underwent laparoscopic feeding gastrostomy at our centre since 2009. RESULTS: Seven children (6 severe generalised recessive dystrophic EB, 1 non-Herlitz junctional EB; 2 girls, 5 boys) underwent modified laparoscopic gastrostomy placement at median age 4.85 years (range 1.0-8.8), with fundoplication for gastro-oesophageal reflux in 1 case, with follow-up for 0.3 to 3.9 years. The procedure was well tolerated with oral feeds usually given after 4 hours and whole protein gastrostomy feeds within 24 hours in 6 patients. Improved growth was reflected in mean weight and height z scores: -1.36 (range -2.6 to 0.5) to -0.61 (range -2.34 to 2.0) and -1.09 (range -2.42 to 1.0) to 0.71 (range -1.86 to 1.0), respectively. Postoperatively, 5 patients experienced minor local complications: minimal leakage without skin damage in 3 and transient peristomal granulation rapidly responsive to topical treatment in 2; this followed acute gastrostomy site infection in 1. There was no leakage after the immediate postoperative period. CONCLUSIONS: We conclude that our less-invasive laparoscopic gastrostomy technique is effective and better tolerated in children with severe EB, at least in the medium term, than open gastrostomy placement. Longer follow-up is required.

Medicated Manuka honey in conservative management of exomphalos major.

PURPOSE: The aim of this study was to assess the effectiveness of Manuka honey ointment and dressings in the conservative management of exomphalos major (EM). METHODS: A retrospective review of five patients with EM who underwent non-operative management with Manuka honey ointments and dressings was carried out to assess the time to complete epithelialisation, time to full feeds, hospital stay, adverse effects, complications and outcome. RESULTS: The skin epithelialisation over the EM sac was achieved in a median of 63 days (48-119). The median time to full enteral feed was 13 days (3-29). The median hospital stay was 66 days (21-121). No adverse effects were noted related to Manuka honey. Three patients had pulmonary hypoplasia requiring prolonged hospitalization; one of those died with respiratory complications at home after achieving complete epithelialisation. The follow-up was a median 16 months (6-22). Two patients did not require repair of the ventral hernia. One patient had ventral hernia repair at 16 months with excellent cosmesis. The remaining patient is awaiting repair. CONCLUSION: This is the first description of the use of medicated Manuka honey ointment and impregnated dressings in the conservative management of EM. This treatment is safe, efficacious and promotes wound healing with favorable outcome.

Retroperitoneal gastric duplication cyst: a case report and literature review.

A rare case of retroperitoneal gastric duplication is reported and discussed. An intra-abdominal cyst was detected at 31 weeks gestation and was followed up prenatally as a left sided duplex kidney. Post-natal ultrasound however, showed a normal kidney, but a cyst with features of enteric duplication in the left upper quadrant adjacent and compressing the kidney. Surgery was carried out during infancy and a retroperitoneal cyst was excised that contained heterotrophic gastric mucosa.

Gastrostomy insertion in the 21st century: PEG or laparoscopic? Report from a large single-centre series.

PURPOSE: To determine whether laparoscopic-assisted gastrostomy (LAG) has superseded percutaneous endoscopic gastrostomy (PEG) based on the clinical outcomes. METHODS: A retrospective study was undertaken for the period January 06-December 09. Demographic and clinical outcomes were recorded and the two groups were compared. RESULTS: 164 patients were studied (PEG, n = 107; LAG, n = 57). 93.5 % of PEG patients required two general anaesthetics compared with 8 % of LAG patients. Median time to using the gastrostomy was 24 (range 0-168) h in PEG and 0 (0-96) h in LAG patients (p < 0.001). Major complications occurred in 15/107 (14 %) of PEG and 2/57 (3.5 %) of LAG patients (p = 0.05). Re-operation rate following complications was 18/107 (16.8 %) for PEG and 3/57 (5.2 %) for LAG (p = 0.05). Minor complications arose in 41/107 (38 %) of PEG and 32/57 (56 %) of LAG (p = 0.05). Post-operative hospital stay was 2 (1-40) days for PEG and 2 (0-20) days for LAG (p = 0.01). The day-case rate was 0/107 for PEG and 5/57 (9 %) for LAG. There was no gastrostomy-related mortality in the series. CONCLUSION: LAG requires fewer anaesthetics, is associated with shorter time to feeding, shortened hospital stay and has a reduced risk of major complications. LAG is a very good alternative to the PEG in children.

A technical innovation in paediatric chest wall reconstruction.

INTRODUCTION: Extensive paediatric chest wall resections and reconstructions are a surgical challenge. Chest wall reconstruction is well known to be associated with chest wall deformity, scoliosis and restrictive lung disease. MATERIALS AND METHODS: We describe, via illustrative cases, the technical innovation of paediatric chest wall reconstruction following tumour resection with titanium rib replacement. RESULTS: The results from this chest wall reconstruction technique have been cosmetically excellent. Both cases, following significant chest wall resections, did not require post-operative ventilation and have returned to normal function. CONCLUSIONS: This Novel Paediatric Chest wall reconstruction technique is ideally suitable after extensive chest wall resection. It has provided stability and protection of underlying viscera and may prevent scoliosis.

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.

Surgical Experience and Learning Points in the Management of Foregut Duplication Cysts.

BACKGROUND: Foregut duplication cysts (FDC) have unpredictable natural course and rarely remain asymptomatic. We present our large cohort of FDC to highlight surgical learning points and outcome. MATERIALS AND METHODS: Review of 41 children with FDC (April 1997-April 2015) included demographics, clinical presentation, preoperative investigations, surgical management, post-operative complications, and overall outcome. RESULTS: Forty-one children (26 girls/15 boys; male/female [M/F] ratio 1:1.7) had 43 FDC (two cases >1 cyst) consisting of antenatally diagnosed 16 (39%), late with symptoms 21 (51.2%), and incidental 4(9.8%) cases. FDC locations were cervical (3), mediastinal (35), and subdiaphragmatic (5). During all cervical FDC resection, recurrent laryngeal nerve was required to be dissected off the cyst wall. Thoracoscopic resection was feasible in 27 cases, with 4 conversions related to infections and adhesions or accidental injury to trachea/esophagus. Complete resection was achieved in all cases with no recurrence. There was no mortality and morbidity included, chylothorax (1) esophageal injury (1), phrenic nerve injury (1), and tracheal injury (2). The minimally invasive surgery and surgery on asymptomatic lesions significantly affect the outcome by reducing need for chest drain (p = 0.01) and length of hospital stay (p = 0.04). CONCLUSION: This retrospective study confirms the efficacy and safety of thoracoscopic surgery; important learning points were association of common wall with trachea in some superior mediastinal cysts and association of recurrent laryngeal nerve in cervical FDC.

Congenital cystic lung lesions: is surgical resection essential?

Can all congenital cystic lung lesions be treated conservatively, without the need for surgery? Our purpose here is to present the morbidity associated with symptomatic cystic lung lesions which have failed to respond to medical treatment. In the past 8 years, 22 consecutive cystic thoracic lesions were retrospectively assessed for clinical presentation, diagnostic modalities, operative findings, technical tribulations, and outcome. The endpoint was complete cessation of recurrent pneumonia and dysphagia. Age at presentation was 7.7 +/- 2.2 years, with 4 +/- 2 episodes per year of lower respiratory tract infection, which had been treated for the past 2.6 +/- 0.3 years. Cough and dyspnea (100%) were the common symptoms, with episodes of cyanosis occurring in 58%. Other significant clinical presentations were dysphagia (55%), failure to thrive (55%), chest pain (46%), haemoptysis (18%), and pleuritic pain (18%). Definitive growth was seen in 91% of the excised specimens. Preoperative morbidity resulted from intractable pneumonia, dysphagia, and failure to thrive. Surgical excision was curative. All 22 children after resection are thriving, with an absence of pneumonia and dysphagia, with normal ventilation/perfusion scans, at 48 +/- 6 months of follow-up. In conclusion, surgical excision of a symptomatic cystic lung lesion that has not responded to medical treatment is recommended.

Pulmonary stabilisation followed by delayed surgery results in favourable outcome in congenital cystic lung lesions with pulmonary hypertension.

OBJECTIVE: Congenital cystic lung lesions associated with fetal hydrops and polyhydramnios are rare, and reported to have greater than 50% mortality, can this be reversed? To propose a period of pulmonary stabilisation and delayed surgery for neonates with congenital cystic lung lesions and pulmonary hypertension. RESULTS: Four neonates with antenatal diagnosed congenital cystic lung lesions with associated fetal hydrops and maternal polyhydramnios, presented with pulmonary hypertension due to lung hypoplasia. Contrast spiral computerised tomography scan was diagnostic. Three had congenital cystic adenomatoid malformation and one extra-lobar pulmonary sequestration with anomalous blood supply from the abdominal aorta. All four were pre-operatively ventilated for 9.8+/-0.9 days on conventional mechanical ventilators. Definitive surgery was performed at 10.8+/-0.8 days following stabilisation of lung function (preductal PO(2) of greater than 60torr with a SaO(2) of 90-100%) and resolution of pulmonary hypertension with absence of persistent fetal circulation on echocardiography. Optimal timing of delayed surgery was in the range of 216-360h. All four are thriving with absent respiratory complications. CONCLUSION: Delayed surgery following pulmonary stabilisation results in favourable outcome.

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.

Paediatric penetrating thoraco-abdominal injury: role of minimallly invasive surgery.

We report two cases of penetrating thoraco-abdominal injuries who presented to our trauma centre. One with stab to lower left chest and the other one had pallet injury to right upper abdomen. The clinical presentation, radiological investigations and operative intervention are reviewed.

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 outcome analysis of paediatric thoracic and cervical neuroblastoma.

OBJECTIVE: To identify factors determining the surgical outcome of primary cervical and thoracic neuroblastoma. METHODS: Twenty-six children with primary thoracic neuroblastoma presented over the last 14 years were analysed for age, mode of presentation, tumour histopathology, biology and outcome. RESULTS: Primary thoracic neuroblastoma was presented in 16 boys and 10 girls at a median age of 2 years (range 6 weeks-15 years). The International Neuroblastoma Staging System (INSS) classified these as Stage 1 (8), Stage 2 (5), Stage 3 (6) and Stage 4 (7). Computed tomography defined the tumour location at the thoracic inlet (11), cervical (2), cervico-thoracic (3), mid-thorax (9) and thoraco-abdominal (1). Twenty-two children underwent surgery that allowed an adequate exposure and resection. Surgical resection was achieved after initial biopsy and preoperative chemotherapy in 15 children, whereas primary resection was performed in 7 children. Four patients with Stage 4 disease underwent chemotherapy alone after initial biopsy; of which, two died despite chemotherapy. Favourable outcome after surgical resection and long-term survival was seen in 19 (86.4%) of the 22 children. Three had local recurrence (14 to 21 months postoperatively), all with unfavourable histology on initial biopsy. The prognostic factors that determined the outcome were age and INSS stage at presentation. In this series, all patients under 2 years of age are still alive, while mortality was seen in five older children. CONCLUSION: Thoracic neuroblastoma in children under 2 years of age irrespective of stage and histology of the tumour results in long-term survival.

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.

Cardiac paraganglioma and gastrointestinal stromal tumor: a pediatric case of Carney-Stratakis syndrome.

The eponymous Carney triad reported the association of gastric leiomyosarcoma, also known as a gastrointestinal stromal tumor, extra-adrenal paraganglioma and pulmonary chondromata. Subsequently, Carney and Stratakis distinguished the inherited gastrointestinal stromal tumor with paraganglioma syndrome from the classical Carney Triad as an autosomal dominant condition in adult patients. This combination of gastrointestinal stromal tumor and cardiac paragangliomata in a child is almost unique.

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.