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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.

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.

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.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).Since 2006 we treated 26 patients with CWD: six were children (age17years, 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).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.

The aim of this study was to assess the effectiveness of Manuka honey ointment and dressings in the conservative management of exomphalos major (EM).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.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.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.

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.

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. Retrospective review of PSP management and outcomes from two UK Tertiary Paediatric hospitals between 2004 and 2015. 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. 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.

In children, the indications for oesophageal substitution are principally, long gap oesophageal atresia (OA), severe anastomotic disruption following primary repair of OA and severe caustic or peptic strictures. We present an outcome review of eight cases who underwent oesophageal substitution with jejunum at our institution between 1986 and 2001. The purpose of this study was to evaluate our experience with free/pedicled jejunal grafts and its long-term outcome as an oesophageal substitute. Operative and postoperative outcome with free and pedicled jejunal grafts in four cases of pure OA, two cases of OA and distal tracheo-oesophageal fistula (TOF), one patient with a high retrolaryngeal oesophageal web and one case of severe caustic oesophageal stricture. Six patients had an oesophagostomy and a gastrostomy fashioned previously. Eleven free jejunal grafts were performed in six patients (three intraoperative redo interpositions for immediate graft loss, three separate grafts in one patient and two free grafts in two patients). One patient's pedicled jejunal graft proximally required microvascular anastomosis while the other had a pedicled graft without microvascular anastomosis. Early postoperative complications included four upper anastomotic leaks (three free grafts, one pedicled with microvascular support), pneumothorax requiring prolonged ventilation and Horner's syndrome. Recurrent laryngeal nerve injury occurred in the patient who had a high retrolaryngeal oesophageal web. During follow up (5-18 years) late complications of upper anastomotic stricture in four patients and graft redundancy with subsequent kinking of the lower anastomosis were observed in one patient. Three patients established a complete oral diet; a further three patients relied on supplemental gastrostomy feeds and one patient is entirely gastrostomy fed. There were two late deaths, one from aspiration and the other from a severe asthmatic attack (5 and 7 months postoperatively, respectively). Our results indicate that there are significant complications related to the use of free jejunal grafts. Early recognition and treatment are of paramount importance in the ultimate achievement of a successful technical outcome.

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 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 ± £2438 vs £6208 ± £2120 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.

Aim The objective of this study is to define the incidence of chromosomal and congenital anomalies in neonates with exomphalos major and minor. Background Incidence of major congenital anomalies varies from 35% to 81% in exomphalos. It is unclear whether these malformations are more common with exomphalos major. Material and Methods The case notes of 82 antenatal diagnoses of exomphalos, made between January 1998 and December 2004, were retrospectively reviewed. Exomphalos major was defined as a defect 5 cm or greater and exomphalos minor a defect less than 5 cm in diameter. Results There were 72 live births, 6 still births, and 4 terminations of pregnancy. There was no statistical significance between exomphalos major and minor regarding mode of delivery, gestational age at birth, birth weight, major cardiac anomalies (21% vs 23%), and renal and external genitalia abnormalities (11% vs 18%). Chromosomal anomalies, syndromes, and dysmorphism were common in exomphalos minor 17 (39%, P = .0001). Congenital malformations of the gastrointestinal tract (14% vs 27%), central nervous system (0 vs 21%), and Wilms' tumor (0 vs 5%) occurred commonly in exomphalos minor. Limb abnormalities (25% vs 5%), ectopia cordis (11% vs 0), and bladder exstrophy (7% vs 0) occurred predominantly in exomphalos major. Mean follow-up was 34 months. Three neonates with exomphalos major died. Overall mortality was 4%. Conclusions Chromosomal anomalies and syndromes occur more commonly in exomphalos minor. Exomphalos minor and major seem to have a predilection for associated anomalies of specific organ systems. This predisposition may help in counseling parents, planning investigations, and organization of multidisciplinary management strategy.

Persistent rectal prolapse is an uncommon but distressing condition in children. Significant controversy exists regarding its surgical management. The aim of this study was to identify a successful management strategy for persistent rectal prolapse in the paediatric population. Records of all children with rectal prolapse treated surgically at Birmingham Children's Hospital between 1995 and 2003 were retrospectively reviewed. Demographic data, clinical presentation, investigations, treatment modality, complications, and outcome were recorded. Inclusion criteria for the study were failure of conservative management leading to operative treatment. An exclusion criterion was cystic fibrosis. A total of 24 patients with persistent rectal prolapse were identified. Two children with cystic fibrosis were excluded from the analysis. Children below the age of 5 years, group I (n=17), were successfully managed by submucous hypertonic saline injections. Eighty-three percent (14/17) were cured by injection sclerotherapy in this group, 12/14 (71%) requiring one injection and 2/14 requiring a second injection. In the three (17.6%) children in group I in whom sclerotherapy failed, cow's milk protein (CMP) allergy was identified as the causative factor. Children older than 5, group II (n=5), either had behavioural problems (n=3) or were autistic (n=2). This group of children with adult-type, full-thickness rectal prolapse were found to be refractory to initial attempts of injection sclerotherapy. All five children were successfully managed with surgical correction. We conclude that rectal submucous hypertonic saline injections are highly effective for managing early-onset idiopathic childhood rectal prolapse. CMP allergy should be considered in young children with recurrent rectal prolapse. We recommend early definitive corrective surgery in older children with persistent rectal prolapse, as they do not respond to conservative measures or injection sclerotherapy.

Objective: The purpose of the study was two-fold: (1) to highlight the varied presentation of mediastinal tuberculous lymphadenitis (MTL) in children and (2) to identify parameters, that may help in the early diagnosis of this condition. Methods: Between January 1995 and December 2002, 13 children with histological diagnosis of MTL were retrospectively assessed for age at presentation, history of exposure to TB, presenting symptoms, investigations, initial diagnosis, surgical treatment and outcome. Stepwise multiple linear regression analysis was used to determine potential risk factors for early diagnosis of MTL. Results: Thirteen children presented with: (a) fever, night sweats and weight loss (4); (b) acute respiratory distress (2); (c) cough and shortness of breath (SOB) (5); (d) stridor (1); and (e) chest pain (1). TB was suspected only in 6 children (46%) at presentation. In the other 7 cases (54%) the presumed diagnoses were: neuroblastoma (nZ1), metastatic malignancy (nZ1), bronchial polyp (nZ1), bronchogenic cyst (nZ2), and presumed foreign body (nZ2). Bronchoscopy was diagnostic in identifying cheesy material within the bronchus and organisms on lavage in 4 (30%) and in identifying external compression in 2 (15%). Thoracotomy and excision of the lymph node mass was necessary to treat the mediastinal compression and to ascertain the diagnosis of TB in 3 children (23%). All 13 children had complete resolution of tuberculous lymphadenitis following anti-tuberculous treatment. The diagnostic clues in this cohort of patients were cough and SOB with history of exposure to tuberculosis (PZ0.0001) and bronchoscopy and lavage with positive staining for acid-fast bacilli (PZ0.0001). Conclusions: Tuberculosis was not suspected in 54% of children with MTL, and they posed diagnostic dilemma on admission. Bronchoscopy must be used as a diagnostic tool in children where tuberculosis cannot be excluded by radiology or specific skin tests. Thoracotomy and excision may be necessary to treat the obstructive symptoms. q 2004 Elsevier B.V. All rights reserved.

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.

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 hadpallet injury to right upper abdomen. The clinical presentation, radiological investigations and operative intervention are reviewed.Key words: Laparoscopy, minimally invasive, penetrating injuries, thoraco-abdominal

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.

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.

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. We describe, via illustrative cases, the technical innovation of paediatric chest wall reconstruction following tumour resection with titanium rib replacement. 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. 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.

In vitro neurite outgrowth and neuronal survival are promoted by laminin, and neuronal migration is promoted by collagen type IV. This led to the hypothesis that Hirschsprung's disease (HD) could be the result of an abnormal extracellular matrix microenvironment in the affected bowel during embryogenesis. Using indirect immunohistochemistry, we studied the distribution of laminin and collagen type IV in the bowel specimens of eight HD patients (four neonates and four infants) and 16 age- and site-matched controls from non-HD patients. In eight HD specimens, the tissue studied was from the aganglionic, transitional, and proximal normoganglionic zones. Uniform distribution of laminin and collagen type IV was observed in the basement membranes of all control specimens. The semiquantitative abnormality in the distribution of these proteins in HD occurred as follows: immunoreactivities were more intense in the inner circular layer, diminishing with a gradient in the outer muscularis externa in six of six aganglionic, four of five transitional, and three of eight normoganglionic sections. The qualitative abnormality of these proteins in HD was speckled immunofluorescence outside the basement membranes of the muscularis externa in all three zones of neonatal specimens only. These findings support the microenvironment hypothesis of the pathogenesis of HD.

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.

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.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.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).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.

Outcome after gastroschisis repair without general anesthesia is controversial, and published conclusions are variable with no comparative studies.The aim of this study was to present a comparative study evaluating outcome after gastroschisis repair with and without general anesthesia.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.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).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.

We report a case of a 4-week-old male infant who presented with increasing respiratory distress and was diagnosed to have bilateral congenital lobar emphysema on chest x-ray. Our approach to the surgical management in two sequential operations of left upper lobectomy followed by right middle lobectomy is discussed.

Spontaneous bronchopleural fistula after childhood empyema remains a surgically challenging condition to treat and is associated with a high morbidity. Four children with pyopneumothorax and associated spontaneous infective bronchopleural fistula are reported. Drainage of the empyema by thoracotomy was performed together with limited decortication and the suturing of a raised digitation of serratus anterior around the fistula to achieve a successful outcome. The surgical technique of raising a serratus anterior digitation flap is described.

Most pediatric thoracic malignancy is pulmonary disease secondary to solid tumors of childhood. The management of isolated pulmonary metastases in adulthood is well documented. Little has been published to document the long-term outcome of pulmonary metastasectomy in childhood. A retrospective study was undertaken to assess the results of surgery for isolated pulmonary metastases. Twenty children underwent surgery over 12 years (mean follow-up 8 years). Five had Wilms’ tumor (mean age 51 months), eight had osteogenic sarcoma (mean age 141 months), three had rhabdomyosarcoma (mean age 92 months), two had hepatoblastoma (mean age 30 months) and two had teratoma (mean age 72 months). Four had bilateral synchronous metastases and thoracotomies, and one had bilateral metachronous metastases and thoracotomies. Nineteen children were discharged well within 10 days of surgery. There was one early complication: a death due to pneumonia. Four children subsequently died postoperatively with cranial metastases (mean 29 months postoperatively). The remaining 16 children remain alive and well. As part of the combined therapy, these results would support an aggressive surgical approach to this disease. Preoperative assessment should include contrast enhanced computed tomogram of the head and chest as well as chest X-ray taken immediately preoperatively to exclude metastases. Bilateral synchronous and metachronous thoracotomy is well tolerated in childhood.

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.

Hirschsprung's disease (congenital colonic aganglionosis) is associated with abnormalities in the distributions and amounts of basement membrane and other extracellular matrix components in the human gut. The authors have investigated the possible significance of nidogen in Hirschsprung's disease, because this glycoprotein is necessary for the formation of ternary complexes with the other basement membrane components, laminin and collagen type IV, and thus may contribute the pathology of the disease. Increased nidogen immunoreactivity in the smooth muscle basement membranes and muscularis mucosae of the distal aganglionic zone in Hirschsprung's bowel was observed, the nidogen immunoreactivity demonstrating that the thickness of the muscularis mucosae was increased in this region. However, steady-state nidogen mRNA levels were found to be significantly lower in both proximal and distal Hirschsprung's bowel (relative to controls). In contrast, no significant differences were observed in the steady-state levels of the mRNAs coding for laminin subunits. These results indicate that although abnormalities in the amount or distribution of nidogen may contribute to the abnormalities seen in the extracellular matrix in Hirschsprung's disease, the levels of expression of the genes coding for either nidogen or laminin are unlikely to be primarily responsible for the lesions.

Fourteen hydrocephalic children with functioning lateral ventricular shunts developed posterior fossa problems: posterior fossa cyst (7 patients); isolated fourth ventricle (6 patients), and concomitant dilatation of third and fourth ventricles (1 patient). Posterior fossa symptoms were present in only 2 children; 10 had nonspecific symptoms and 2 were asymptomatic. Computed tomography was the most useful investigation for diagnosis and management. Decompression of progressing posterior fossa lesions was attempted in 5 patients: cystoperitoneal shunt (3), marsupialization (1), and aspiration (1). All 5 patients had initial symptomatic relief but symptoms recurred subsequently; 2 eventually died of severe ventriculitis. The remaining 9 did not require posterior fossa surgery.

Heterotopic gastric mucosa is reported in a sinus present since birth in the midline of the anterior two thirds of the tongue. Current theories concerning the presence of heterotopic mucosa in the oral cavity are presented and discussed.