Long-term functional outcome of sacrococcygeal teratoma in a UK regional center (1993 to 2006).

BACKGROUND: Sacrococcygeal teratoma (SCT) is the commonest neonatal neoplasm. Its long-term effects are important in prenatal counseling and the delivery of an appropriate postoperative plan. AIM: To determine the long-term functional outcome after SCT excision in a UK regional center. STUDY DESIGN: Follow-up data for all patients with a SCT excised at the John Radcliffe Hospital in Oxford was collected retrospectively from notes and prospectively in clinic visits. OUTCOME MEASURES: Clinical evidence of bowel or bladder impairment, mortality. RESULTS: Over a 14-year period, 18 patients had a histologic diagnosis of SCT. Nine patients (50%) were born, 7 (39%) were terminated, and 2 (11%) were stillbirths. Of the 9 patients who had SCT resection, 4 (44%) were antenatally diagnosed. There were no perioperative deaths and alpha-fetoprotein levels normalized by 6 to 12 months after tumor resection. Median follow-up of patients was 30 months (range: 6 to 132 mo) with 1 patient lost to follow-up at 6 months, although he was asymptomatic at the time. Three patients developed urologic complications (2 within 1 year of tumor resection), including 2 patients with neurogenic bladder dysfunction and 1 patient with detrusor sphincter dyssynergia. CONCLUSIONS: Approximately one-third of patients will develop major urologic complications after resection of SCT. Routine ultrasonography in the first postoperative year after tumor resection may help to identify patients with neuropathic bladder at the early stage and predict late complications. Parents need to be aware of this potential long-term complication during prenatal counseling and the need for regular long-term follow-up with the pediatric surgical team.

Fibroepithelial polyps causing ureteropelvic junction obstruction in children--a case report and review article.

Fibroepithelial polyps are extremely rare benign mesodermal tumours in children that can cause ureteropelvic junction (UPJ) obstruction. We report on a 10-year-old boy presenting with UPJ obstruction due to a fibroepithelial polyp, and review 28 similar published paediatric cases.

The management of renal conditions in the perinatal period.

Most urogenital abnormalities are now diagnosed antenatally on high resolution ultrasound scans. This has enabled recognition of those that are not compatible with survival and these are managed with termination of pregnancy. Renal anomalies that require surgical intervention continue to pose challenges. Conditions such as multicystic dysplastic kidney can be easily recognised and managed based on the experience gained with long-term studies of its natural history. Polycystic kidney on the other hand while not posing a diagnostic problem remains beyond the reach of therapeutic intervention and postnatal supportive measures are the only available means of dealing with this entity at present. The major difficulty is with the management of antenatally diagnosed pelvicalyceal dilatation. The goal of intervention is to preserve renal function when dilatation is the consequence of obstruction. Unfortunately, by the time ultrasound evidence of significant obstruction is apparent renal damage is already established. Fetal intervention should be considered in those cases where severe oligohydramnios is associated with hydronephrosis, especially in the presence of a solitary kidney or in bilateral disease. Postnatally, all neonates with renal tract dilatation should be managed according to a protocol which mandates serial measurements of renal pelvis diameter and correlates this with data from radionuclide scans. This will enable recognition of kidneys that are at risk of losing function while at the same time avoiding unnecessary surgical intervention in those which remain dilated but are functionally stable.

Acute retention of urine due to prolapsing stoma in a case of anorectal malformation with bladder neck fistula.

This case report describes an extremely premature infant who was born with a high anorectal malformation requiring a colostomy soon after birth. He later developed multiple episodes of acute urinary retention complicated by bilateral hydronephrosis and acute renal failure. The cause of the retention was found to be the prolapsing stoma, which was kinking the bladder neck.

When is a mediastinal mass critical in a child? An analysis of 29 patients.

The aims of this study were to determine the pattern of presentation of childhood mediastinal masses in our community and to identify factors associated with the development of acute airway compromise. The authors retrospectively reviewed the records of 29 consecutive patients with mediastinal masses managed at their institution between January 1995 and December 2001. Demographic data, mass characteristics, clinical presentation, and surgical procedures were recorded. Seven patients (24.1%) were asymptomatic at presentation. Eight (27.6%) were classified as having acute airway compromise at presentation. Respiratory symptoms and signs were the most common mode of presentation (58.6% and 55.2%, respectively). The most common histological diagnosis was neurogenic mass (37.9%), followed by lymphoma (24.1%). Most masses were located in the superior mediastinum (41.1%). Factors associated with the development of acute airway compromise were (1) anterior location of the mediastinal mass (P=0.019), (2) histological diagnosis of lymphoma (P=0.008), (3) symptoms and signs of superior vena cava syndrome (P=0.015 and 0.003, respectively), (4) radiological evidence of vessel compression or displacement (P=0.015), (5) pericardial effusion (P=0.015), and (6) pleural effusion (P=0.033). Clinical presentation of childhood mediastinal masses is often nonspecific or incidental. Yet they have the propensity of developing acute airway compromise, which is closely associated with superior vena cava obstruction. Such patients should be managed as a complex cardiorespiratory syndrome, termed "critical mediastinal mass syndrome", by an experienced multidisciplinary team.

Carbon dioxide laser circumcisions for children.

The carbon dioxide laser for circumcision was introduced by our department in 1989. This study aims to review our experience with laser circumcision for children and to evaluate its cost effectiveness as compared to conventional methods. A retrospective study of 30 patients who underwent conventional circumcision in 1985 and another 30 patients who underwent laser circumcision in 1995 was undertaken. The operating times in both groups were compared. The total cost of use of the laser machine was calculated, taking into account maintenance costs, estimated life span of laser machines (10 years) and costs of disposables used during each circumcision. This was weighed against the cost savings from shorter operating times and reduced operating theatre facility charges. Also, morbidity data from 2781 laser circumcisions done between May 1997 and April 2000 was collected. There was a significant decrease of 5 minutes in operating time for the group of patients who underwent laser circumcision. Calculated cost savings per laser circumcision from the reduced operating theatre time was S dollars 31/-. Of the 2781 cases of laser circumcision performed, there was an overall complication rate of 1.15%. Twenty-nine cases (1.04%) had post circumcision bleeding, of which 10 cases (0.36%) required unplanned return to operating theatre for hemostasis. Three cases (0.11%) had wound infection, requiring admission to hospital. Laser circumcision is a simple method with reduced operative time translating into cost effectiveness. Morbidity rates of laser circumcision compare favourably to those of conventional circumcision based on reports from other institutions.

Pleuropulmonary blastoma with a large embolic cerebral infarct.

We report on a 3-year-old girl who developed a large embolic cerebral infarct 1 day after an uneventful thoracotomy to remove a large pleuropulmonary blastoma. The tumour had encased the heart and great vessels and ruptured into the left hemithorax. Pleuropulmonary blastoma is a rare, but unique, primary thoracic neoplasm in young children and, to our knowledge, the development of a secondary large embolic cerebral infarct is also uncommon and has not been reported in this tumour.

One-stage surgical correction of proximal hypospadias.

INTRODUCTION: Proximal hypospadias poses major problems in surgical correction owing to the complexity and severity of the abnormalities, leading to the use of staged repairs to correct this condition. However, with precise definition of the components of this deformity a single-stage operation can be developed and applied successfully for surgical correction of this condition. MATERIALS AND METHODS: Twenty-six patients with severe proximal hypospadias were subjected to a one-stage repair. Excision of proximal fibrotic dartos tissue and removal of dysplastic urethral plate tissue corrected chordee completely. Urethral reconstruction was then performed by tubularising a flap of dorso-lateral preputial skin which was then anastomosed to the proximal urethra. The glandular part of the urethra was reconstructed using the distal part of the flap as an onlay graft over the meatal groove. The suture lines were covered with a layer of dartos tissue and skin closure was completed by transposing dorsal skin to surface the ventral penile shaft. A urethral catheter was left in for 10 days. RESULTS: All patients have been followed up after surgery from 1 to 5 years with a median period of 2 years. There were no fistulas. Two patients had mild stenosis at the meatus which responded to dilatation. One patient developed a stricture at the proximal anastomosis which required secondary correction. All other patients achieved satisfactory correction, both in terms of voiding and in the cosmetic appearance of the genitalia. CONCLUSION: Single-stage repair of hypospadias can be successfully applied in the correction of severe proximal hypospadias. It requires meticulous dissection and careful design of reconstructive techniques. The end results are comparable to staged procedures and morbidity is significantly lower.

Comparing wound closure using tissue glue versus subcuticular suture for pediatric surgical incisions: a prospective, randomised trial.

Tissue adhesives have gained favour for quicker and painless closure of lacerations. To compare the tissue adhesive 2-octylcyanoacrylate with our current standard subcuticular suture for closure of surgical incisions in children, looking at outcome measures of time efficiency, cosmesis, and wound complications, a prospective, randomised, controlled trial was conducted at our institution's ambulatory surgery centre. All healthy patients undergoing unilateral or bilateral herniotomies were recruited prospectively with informed consent and randomly allocated to suture or glue. The exclusion criteria were neonates or children with allergy to tissue glue. Time of wound closure was measured from the subcutaneous layer to application of the dressing. An independent, blinded observer assessed cosmesis at 2 to 3 weeks using a validated wound scale ranging from worst (0) to best (6). Parent satisfaction with wound appearance was recorded on a 100-mm visual analogue scale (VAS). A total of 59 patients were recruited into the study with 26 in the glue group and 33 in the suture group. There was no difference in mean time of closure (glue 181 +/- 62 s vs suture 161 +/- 45 s, P = 0.18). Two patients in each group had a suboptimal Hollander wound score of 5 (7.7% glue, 6.1% suture). There was also no difference in parent satisfaction (VAS: glue 78 +/- 19 mm vs suture 81 +/- 15 mm, P = 0.68). No patient reported any rash, wound infection, or dehiscence. Tissue glue is easy to use with no complications and has equivalent cosmetic results, but is not faster than a subcuticular suture.

Clinics in diagnostic imaging (61). Left primary megaureter with lower ureteric stone.

Megaureter is infrequently diagnosed in adulthood. A 27-year-old man who presented with recurrent left loin pain was found to have megaureter on intravenous urography. His symptoms did not resolve after balloon dilatation of the 3 cm stenotic aperistaltic segment of the lower ureter. He eventually underwent ureteric re-implantation with satisfactory symptomatic relief. The resolution of obstruction was demonstrated radiologically. The role of imaging in the diagnosis and surveillance of megaureter, as well as indications for intervention, are discussed.

Experience with video-assisted thoracoscopic surgery in the management of complicated pneumonia in children.

PURPOSE: The aim of this study was to assess the impact of video assisted thoracoscopic surgery (VATS) in the management of empyema in children. METHODS: This report involves cases of complicated pneumonia in children requiring surgical intervention after failure of medical treatment with antibiotics, with or without drainage from November 1997 to October 1999. The impact of VATS has been studied prospectively from October 1998 when VATS was introduced. The results have been compared with the previous year when similar cases were dealt with open thoracotomy. These 2 groups of patients with VATS (V) or without VATS (O) were studied for their progress in hospital and the final outcome. RESULTS: A total of 39 immunocompetent children with community-acquired pneumonia were studied. There were 17 cases in O and 22 in V. There were 2 conversions to open thoracotomy in V. Both of these cases required resection of the lung parenchyma for severe necrosis and bronchopleural fistula. The mean age in years was 5.3 (O) and 4.9 (V). Parameters that were significantly less in V compared with O include timing of referral (O, 13.6 days; V, 5.3 days), number of lung resections (O, 8; V, 2), blood transfusion (O, 14; V, 2), analgesia requirements (O, 7.8 days; V, 2.9 days), postoperative length of stay in hospital (O, 10.4 days; V, 4.6 days), time to become normothermic (O, 5.6 days; V, 1.7 days); and time to removal of chest drains (O, 6.0 days; V, 2.7 days). Cosmesis is superior in cases of VATS compared with open thoracotomy. All the children recovered well on follow-up with resolution of symptoms and no recurrences. CONCLUSIONS: (1) VATS has ushered in a new era of hope for patients with complicated pneumonia. (2) Thoracotomy, lung resections, and the attending morbidity rate have decreased. (3) Patients are being referred earlier by the physicians because the management protocol is changing.

Salmonella typhimurium: a rare cause of colonic ulceration and perforation in infancy.

A rare case of a healthy infant with colonic ulcers caused by Salmonella typhimurium infection that presented with colonic perforation, hypovolemic, and septicemic shock is discussed. It stresses the importance of considering an infective process such as salmonellosis in the differential diagnosis of colonic ulceration in an infant and illustrates the unique histologic finding of colonic inflammatory changes with sparing of the small intestine.

A combined tubularized/onlay graft technique for total correction of severe hypospadias.

BACKGROUND/PURPOSE: A combined tubularized/onlay graft technique is described for the complete correction of chordee with urethroplasty in a single stage in cases of severe hypospadias. METHODS: Twenty-two patients with severe hypospadias ranging in age from 9 months to 11 years underwent single-stage correction using a technique developed by the author. In this method, chordee is first completely excised by removing all fibrotic tissue both proximal and distal to the urethral orifice, preserving the meatal groove. A dorsolateral preputial flap is then raised and tubularized to form the neourethra. The proximal end of this tube is anastomosed to the urethral opening using a continuous absorbable suture. Two parallel incisions are made in the glans on either side of the meatal groove. The distal part of the neourethral flap is laid over the groove and sutured on either side to create the glanular part of the urethra, after which the glans is reconstructed with the new meatal opening at the tip. The neourethral suture line is covered with a layer of vascularized subcutaneous tissue to protect against fistula formation, and the rest of the preputial skin is transferred ventrally to provide cover for the penile shaft. RESULTS: There were no major complications with minimum follow-up of 20 months. Meatal stenosis developed in two patients, and one had stricture at the proximal anastomosis. These were treated successfully with minor corrective procedures. All other patients had good results, and there were no cases of fistula. CONCLUSIONS: The method described has proved successful in the surgical correction of severe hypospadias in a single stage. It is easily adapted to permit urethral reconstruction after varying degrees of tissue excision required to obtain satisfactory correction of chordee. Patients do not need to undergo multiple procedures, and no major complications were encountered in this series.

The viability of liver graft for transplantation after prolonged warm ischaemia.

Maximum duration of warm ischaemia within which the liver graft is viable for transplantation remains undefined. Published data on porcine allogeneic liver transplantation (LTx) using non heart beating donors (NHBDs) are conflicting because technical details like the hepatic artery status, systemic heparinisation of donor animals and duration of rewarming were not addressed. We described a novel porcine model which simulate conditions of transplantation from NHBDs. The pigs were divided into three groups of 6 each. Groups I, II and III were subjected to 60, 90 and 120 minutes of warm ischaemia, respectively. Liver viability was assessed using four parameters: serum liver function tests (serum bilirubin and transaminase), dynamic liver function test i.e. the monoethylglycinexylidide (MEGX) formation test, morphological assessment and animal survival. All animals in groups I and II (90 minutes of warm ischaemia or less) survived but 50% of animals in group III died of massive liver failure. Given that rewarming period required in actual allogeneic LTx is about 60 minutes, the safe period for intervention in NHBDs is determined to be about 30 minutes. Allogeneic porcine LTx using NHBDs with 30 minutes of cardiac arrest were performed in 5 animals. All of them survived.

Pudendal-thigh flap vaginoplasty in the reconstruction of genital anomalies.

Children with abnormal genitalia associated with intersex anomalies or cloacal malformations require vaginal reconstruction. Although many procedures using skin grafts, skin flaps, and intestinal segments have been described, they all have disadvantages. In the present study 12 patients with genital defects who required vaginoplasty underwent surgery using a new technique using bilateral pudendal-thigh flaps based on the posterior labial artery. There were six patients with congenital adrenal hyperplasia (CAH), three with vaginal atresia, two with cloacal deformities, and one with testicular feminizing syndrome. Four of the CAH patients and the two with cloaca underwent vaginoplasty as second-stage procedures. All other cases were treated with one-stage operations. This method of vaginoplasty can be combined with correction of other associated abnormalities, and it has proved to be simple and reliable with satisfactory functional and cosmetic results.

Mortality among infants with high-risk congenital diaphragmatic hernia in Singapore.

Several factors suggested to predict mortality in congenital diaphragmatic hernia (CDH) have not always been applicable in different centers. A retrospective review was conducted of 19 consecutive neonates in Singapore in whom CDH was diagnosed within 12 hours of birth to identify factors associated with mortality. Of the 19 cases, 15 (79%) were diagnosed using antenatal ultrasonography. Eight (42%) underwent primary repair at a median age of 23 hours (range, 12 to 50 hours). Of the 19 infants, 15 died (mortality rate, 79%). Survivors until hospital discharge were compared with nonsurvivors. Antenatal diagnosis and stomach position in left-sided defects had no effect on outcome, although polyhydramnios tended to be associated with nonsurvival. Significant postnatal factors associated with mortality included a low arterial pH level, low initial arterial-alveolar oxygen ratio, high initial alveolar-arterial oxygen gradient, as well as high oxygenation and ventilation indices. These results reflect difficulty in oxygenation because of pulmonary hypoplasia despite evidence of adequate ventilation. There was no difference between survivors and nonsurvivors in either their initial or best postductal blood gases. The "Bohn quadrants" did not aid in predicting survival of infants who underwent repair because all eight such infants had best postductal carbon dioxide values of less than 40 mm Hg and ventilation indices of less than 1,000. Yet only four (50%) survived until hospital discharge. Large-scale evaluation of these factors may be required in the future to demonstrate their validity and reliability because of changing management strategies for CDH.

A new approach to the surgical correction of buried penis.

Buried penis has been variously attributed to obesity with excessive suprapubic fat, severe phimosis with trapping of the penis within the prepubic tissues, and inadequate fixation of the penile shaft skin at the base resulting in tenting. Previous attempts at surgical correction, by excising suprapubic fat, fixing penile shaft skin to the base of the penis, and circumcising, have failed to give satisfactory results and, indeed, procedures like circumcision will make the condition even worse. The technique developed by the author is based on the recognition that this condition exists because of the displacement of the root of the penis below its normal position, resulting in the surrounding fat and dartos tissues enveloping the penile shaft. In this procedure, dissection at the root of the penis is carried out deep down to the corporal bodies. All fibrotic tissue that binds the penile shaft is excised. The lengthened penile shaft is anchored at its base by suturing the surrounding tissue onto the tunica. This technique has been applied in 22 patients ranging in age from 5 months to 11 years. Apart from two technical problems, all other patients had satisfactory correction with good functional results.

Single stage excision of preauricular sinus.

A total of 44 patients with preauricular sinus underwent one-stage surgical treatment. There were 32 unilateral and 12 bilateral cases giving a total of 56 sinuses. Of these 16 were infected at the time of presentation. Based on the observation that preauricular sinus represents the duct of a preauricular gland which is closely adherent to the fibrocartilage of the ear, the authors propose a method for the complete cure of this lesion by excising the whole gland and duct together. This technique was used in all cases including 16 patients with infected sinuses. There were three recurrences, two of which were due to incomplete excision of the gland. The third case was found to have a fistula leading to an atretic external auditory canal. All other patients have shown no evidence of recurrence on follow up of six months or longer. Our experience has shown that preauricular sinus can be effectively treated at any stage by a simple surgical technique based on a clear understanding of the underlying anatomy of the lesion.

Congenital tracheobiliary fistula.

A case of congenital tracheobiliary fistula with absent common bile duct is reported. The patient presented with repeated aspiration pneumonia, and the diagnosis was established during bronchoscopy. Excision of the fistula and cholecystoduodenostomy were successful. This is the fourth reported case of its type and the first one to survive surgical treatment. The condition is a rare cause of respiratory distress in neonates and should be considered in the differential diagnosis of intractable aspiration pneumonia.