Subtle Radiological Features of Splenic Avulsion following Abdominal Trauma.

Splenic trauma in children following blunt abdominal injury is usually treated by nonoperative management (NOM). Splenectomy following abdominal trauma is rare in children. NOM is successful as in the majority of instances the injury to the spleen is contained within its capsule or a localised haematoma. Rarely, the spleen may suffer from an avulsion injury that causes severe uncontrollable bleeding and necessitates an emergency laparotomy and splenectomy. We report two cases of children requiring splenectomy following severe blunt abdominal injury. In both instances emergency laparotomy was undertaken for uncontrollable bleeding despite resuscitation. The operating team was unaware of the precise source of bleeding preoperatively. Retrospective review of the computed tomography (CT) scans revealed subtle radiological features that indicate splenic avulsion. We wish to highlight these radiological features of splenic avulsion as they can help to focus management decisions regarding the need/timing for a laparotomy following blunt abdominal trauma in children.

Micturating cystography and "double urethral catheter technique" to define the anatomy of anorectal malformations.

Ano-rectal malformations (ARM) in the male patient may be associated with a fistulous communication between the rectum and urethra. Pre-operative radiological assessment is important to delineate (a) the presence and level of the fistula, (b) the anatomy of the posterior urethra and (c) any anomalies in adjacent structures. Bladder catheterisation can be technically difficult when performing an MCUG and distal loopogram in such patients. This can be due to urethral stricture, tortuous or kinked urethra or preferential passage of catheter into a large fistula and leads to an inadequate study. We describe a "double urethral catheter technique" to enable urethral catheterisation when the fistula is large.

Wilms' tumour with persistent intravascular extension: a review of the surgical aspects of management.

AIM: To review the surgical management of Wilms' tumour with persistent intravascular (vena caval +/- atrial) tumour extension. PATIENTS AND METHODS: Data were collected regarding operative details, tumour and 'thrombus' histology, and long-term outcome for patients with Wilms' tumour with cavo-artial extension. RESULT: From 1988 to 2004, 13 patients underwent treatment for Wilms' tumour with persistent intravascular extension. Preoperative chemotherapy was administered in 11/13 patients and postoperative radiotherapy in eight patients. Intravascular involvement was upto IVC (5), and right atrium (8) patients. Techniques employed for excision of intra-vascular component were: local cavotomy (3), extensive infra-diaphragmatic cavotomy without cardiopulmonary bypass (CPB) (1), and excision of cavo-atrial tumour with CPB (+/- hypothermia and cardiac arrest) (9). Mean time on CPB was 90 min. Caval repair was accomplished by primary repair (6) and pericardial graft in (7) patients. There were no intraoperative deaths and few major complications. Tumour thrombus contained malignant cells in 10/13 cases. Mean follow up has been for 55.4 months. To date, seven patients remain disease-free (one lost to follow up), disease recurred in five patients, three of whom have died. There were no symptoms related to the graft. CONCLUSIONS: Surgery for Wilms' tumour with persistent intravascular extension despite chemotherapy is technically challenging. CPB +/- hypothermia and cardiac arrest and extensive caval repair with a graft is safe and reliable in the long term.

Primary intrarenal neuroblastoma: a rare, aggressive tumour of childhood mimicking Wilms' tumour.

Primary intrarenal neuroblastoma is an extremely rare and aggressive tumour of the kidney. We report on a case that mimicked Wilms' tumour. Differentiating primary intrarenal neuroblastoma from Wilms' tumour is important both for management and prognosis.

Pyloric stenosis: unusual presentations and rare assoclations.

Pyloric stenosis is a common paediatric surgical condition with a well-recognised pattern of clinical features. Diagnosis is usually straightforward and a Ramstedt pyloromyotomy is curative. We present three patients who developed pyloric stenosis incidentally during the management of other primary surgical conditions. Issues of diagnostic difficulty following presentation of pyloric stenosis in the post-operative period (all three patients), its rare syndromic associations (one patient) and its management in this unusual situation are discussed. An awareness of the unusual presentations of pyloric stenosis during treatment for other primary surgical conditions can help in earlier diagnosis and treatment.

Chromosomal translocation in mesenchymal hamartoma of liver: what is its significance?

Mesenchymal hamartoma of the liver is generally considered to be benign in nature, and surgical excision has been the treatment of choice. There have been 3 reports on the association of cytogenetic abnormalities with this tumor, and the possibility of malignant potential has been raised. The authors report a fourth case with a previously unreported complex chromosomal translocation between chromosomes 11, 17, and 19 at bands q12, p11, and q13.3, respectively in all cells examined and discuss the significance of this finding.

Congenital cystic mesoblastic nephroma.

With widespread use of antenatal ultrasound, an increasing number of urinary-tract lesions are being detected, and the clinician is called upon to make management decisions. Parenchymal lesions of the kidney pose a special challenge because of the associated risk of the occurrence of malignancy. A case of an antenatally-detected solid/cystic lesion of the kidney diagnosed as a congenital cystic mesoblastic nephroma following nephrectomy is presented and the issues involved in therapeutic decision-making are discussed.

Preoperative enterocolitis is associated with poorer long-term bowel function after Soave-Boley endorectal pull-through for Hirschsprung's disease.

BACKGROUND/PURPOSE: The purpose of the investigation was to apply a semiquantitative scoring system for bowel function to patients who had undergone endorectal pull-through (ERPT) for Hirschsprung's disease (HD) and to use this to analyse the clinical factors relating to functional outcome. METHODS: The case note details of 63 patients undergoing ERPT for HD were reviewed. A questionnaire using a semiquantitative scoring system for 6 aspects of bowel function was sent to parents of 55 patients. The total functional score (TFS) for each patient was calculated, and a statistical analysis was performed to determine which clinical factors were significantly related to bowel functional outcome. RESULTS: Fifty (91%) of the parents returned the questionnaire. TFS was "good" in 45%, "fair" in 33% and "poor" in 22%. There was a statistically significant difference in the scores of those who had preoperative Hirschsprung's associated enterocolitis (HAEC) TFS = 8.0 and those who did not, TFS 12.7 (P <.01) The sex of the patient, length of aganglionic segment, timing of ERPT (early/late), staging of ERPT (1 or 2), presence/absence of anastomotic stricture, and presence or absence of Down's syndrome did not statistically significantly affect TFS. CONCLUSIONS: Preoperative HAEC was the most important factor in relation to functional outcome after ERPT for HD. The reason remains unclear.

Perianal abscess in childhood.

Perianal abscess (PA) is a common condition encountered in childhood, but its optimal primary treatment is uncertain. Treatment of PA by incision and drainage (I & D) alone is associated with an unacceptably high recurrence rate, either as fistula-in-ano (FIA) or as PA. To identify possible causes of recurrence and assess the value of concomitant laying open of a fistulous tract at the time of primary incision and drainage, the case notes of all children who presented to our institution with a PA between January 1992 and January 1997 were reviewed retrospectively. Thirty-three cases were identified (29 boys and 4 girls). A fistulous tract was identified and laid open at the time of primary drainage in 20 cases, whilst 13 were treated by I & D alone. Following primary drainage, there were 7 recurrences (21.2%) (FIA 5 and PA 2). All recurrences had been primarily treated by drainage alone, whilst there were no recurrences in patients who had also undergone fistulotomy at the time of primary drainage. Thus the primary treatment of PA in childhood should involve a careful search for a coexisting fistula and treatment of this by fistulotomy.

Management of simple renal cysts in children.

Simple renal cysts are uncommon in children and their presentation and management has changed with increasing use of ultrasound scans. The aim of this study was to review our experience and highlight some peculiarities in diagnosis and management of these cases. Eight cases were diagnosed and two symptomatic cases underwent aspiration under ultrasound guidance; one case recurred and required re-aspiration. Differentiation of simple renal cysts from other cystic lesions of the kidney, aspiration of symptomatic cysts and the importance of long-term follow-up are discussed.

Superior mesenteric artery syndrome in children.

Superior mesenteric artery syndrome is an uncommon clinical condition characterised by features of acute or chronic upper gastrointestinal tract obstruction. It sometimes has particular clinical associations such as weight loss, spinal surgery cast application or adhesions. Associated multiple clinical problems can complicate the picture and delay diagnosis. Diagnosis is usually by barium meal showing compression of the 3rd part of duodenum. Treatment can be conservative or surgical. Surgical treatment consists of division of the ligament of Treitz and derotation of the gut. We review our experience of four children with this syndrome.