Xanthogranulomatous pyelonephritis in childhood.

BACKGROUND: Xanthogranulomatous pyelonephritis is a severe, atypical form of chronic renal parenchymal infection accounting for 6/1000 surgically proved cases of chronic pyelonephritis. Its manifestations mimic those of neoplastic and other inflammatory renal parenchymal diseases and, consequently, it is often misdiagnosed preoperatively. AIM: To examine the relation between clinical history and the results of renal investigations performed in children with xanthogranulomatous pyelonephritis. METHOD: A retrospective review of 31 cases presenting with the histopathological diagnosis of xanthogranulomatous pyelonephritis between 1963 and 1999. RESULTS: The mean follow up was 8.2 years. The male:female ratio was 1:1.1. The left kidney was affected in 26 of the 31 patients. The positive findings on examination and investigation at presentation were: fever, 16 children; pyuria, 26 children; positive urine culture, 16 children. A haemoglobin of < 100 g/l was measured in 27 of 31 patients and 15 of 18 patients tested had a raised erythrocyte sedimentation rate of > 20 mm in the first hour. Twenty six children had renal calculi, with a large reduction in the function of the affected kidney on isotope scintigraphy in 27 of the 29 patients tested. Hypertrophy of the contralateral kidney, shown on imaging, was present in 17 of 31 patients. CONCLUSIONS: Increasing awareness of this condition should lead to the diagnosis being suspected preoperatively.

Splenic cysts--aspiration or partial splenic decapsulation?

We report on 2 children who had partial splenic decapsulation for splenic cysts--a pseudocyst and a congenital epidermoid cyst. Ultrasound-guided percutaneous needle aspiration with drainage was attempted in both patients but resulted in rapid cyst reaccumulation. Used on its own in the management of splenic cysts, percutaneous drainage has limited therapeutic value and delays definitive surgery. Partial splenic decapsulation is a simple and safe procedure.

Emergency sleeve lobectomy after blunt chest trauma in a child.

The clinical features and successful management of a patient with right main bronchus disruption after blunt chest trauma are described. The presentation was one of bilateral tension pneumothoraces. A high index of suspicion, coupled with appropriate airway management at presentation, was vital for the successful treatment of this patient. Surgical resection using a sleeve lobectomy, an operation rarely used in trauma patients, was highly effective in this patient, and the technique is described in this report.

Normal and abnormal pelviscopic anatomy at the internal inguinal ring in boys and the vasal triangle.

OBJECTIVES: We sought to explain and define normal and abnormal laparoscopic pelvic anatomy, which has only recently become the target of much attention. METHODS: The embryology, normal anatomic landmarks, and abnormal findings of the male pelvis, as discerned from more than 350 laparoscopic investigations in boys, were analyzed. RESULTS: The medial umbilical ligament, the wishbone at the internal inguinal ring, the transverse vesical fold, and the vasal triangle are principal laparoscopic landmarks of the male pelvis. Deficient spermatic vessels, abnormal gonadal locations, patent processus vaginalis, single medial umbilical ligament, and transverse testicular ectopia were the abnormal findings. CONCLUSIONS: Laparoscopic familiarity with the male pelvis permits safe and efficient diagnostic and therapeutic navigation in this new surgical arena.

Long term outcome after surgery for extrahepatic portal vein thrombosis.

The long term outcome of 21 children with extrahepatic portal hypertension secondary to portal vein thrombosis managed by surgical intervention was evaluated. Portosystemic shunts, used primarily in nine patients (eight central splenorenal, one mesocaval) after conservative treatment had failed, had no associated mortality and a 56% patency rate. Five of these shunted patients had no further bleeding episodes and did not show encephalopathic impairment. Direct attack procedures-portoazygos operation (four patients) was associated with significant complications, including one fatality. Other direct approaches-oesophageal transection and variceal plication (five patients) had variable outcome. Splenectomy alone (three patients) ameliorated hypersplenism; however, further surgery for recurrent haemorrhage (two patients) was necessary. Endoscopic sclerotherapy controlled recurrent variceal bleeding (three patients) when it became available to the unit. Conservative treatment practised in five children had little success: two patients survived, two died from further haemorrhage, and one was lost to follow up. These results suggest that in centres without endoscopic expertise, and for patients who are sclerotherapy 'failures', surgery can be performed safely and achieve reasonable long term success rates in childhood extrahepatic portal hypertension.

The effect of two-stage orchiopexy on testicular growth.

Testicular growth was measured in 33 males who had undergone 2 orchiopexies on the developing testis. Multiple surgery on the abdominal or canalicular testis was associated with a high rate of testicular atrophy (40%) and a 46% decrease in testicular volume. A similar decrease (43%) in testicular volume was noted in those patients with an infracanalicular testis but no testis atrophied in this group. The results suggest that primary orchiectomy be considered in the high testis provided the contralateral testis is normal, or alternatively a Fowler-Stephens or microvascular transplant if it is not. Patients with an infracanalicular testis may undergo a careful second-stage orchiopexy. All patients with retarded testicular growth should perform routine self-examination.

Laryngo-tracheo-oesophageal cleft: a plea for early diagnosis.

Congenital laryngeal clefts are rare. This paper reports on the management of 3 patients with Type 2 laryngo-tracheo-oesophageal cleft. One patient died following tracheopexy after successful cleft closure. Prognosis is related to the presence of other major anomalies, the early diagnosis of the lesion and institution of appropriate respiratory and nutritional care prior to correction of the defect. A greater awareness of the condition combined with aggressive diagnostic endoscopy should result in early diagnosis and improved survival.

Ureteric cyst: an unusual abdominal mass.

Abdominal cystic masses in childhood are rare and despite improved imaging techniques continue to present diagnostic confusion. Typically such cystic lesions are mesenteric or intestinal in origin. However, rarer causes include cystic dilatation of a blind ending ureter. To date over 100 cases of blind ending ureters have been reported and their management described. We report on a case of non-communicating ureteric cyst associated with a blind ending ureter that recurred following percutaneous drainage and required operative resection. Although percutaneous drainage techniques have been advocated for a variety of abdominal cystic lesions, the experience gained from this case suggests that operative excision is the treatment of choice.

Nissen fundoplication for gastro-esophageal reflux in repaired tracheo-esophageal fistula.

One hundred and fifty three patients had repair of tracheo-oesophageal fistula in a 15 year period of observation. Of these 55 had associated gastro-oesophageal reflux and of these 12 (8%) underwent a Nissen fundoplication. All patients manifested significant failure to thrive but weight-gain was documented in all patients following their fundoplication. Twenty five percent of patients demonstrated recurrent reflux in this study and emphasises the difficulty of treating gastro-oesophageal reflux in repaired tracheo-oesophageal fistula patients. Careful selection is important.

Delayed primary anastomosis for esophageal atresia: 18 months' to 11 years' follow-up.

In 1977 we started treating babies with isolated esophageal atresia by delayed primary anastomosis and in 1981 reported our early experience in five cases treated between 1977 and 1979. Since 1979, 11 further consecutive cases have been managed by initial gastrostomy followed by delayed primary esophageal anastomosis. Their mean gestation was 35 weeks (range, 28 to 40 weeks) and mean birth weight was 2,040 g (range, 1,140 to 2,720 g). The esophageal gap between the two ends when assessed initially at fluoroscopy ranged from 2.2 to 4.5 cm (mean, 3.2 cm). Age at delayed primary anastomosis ranged from 6 to 20 weeks. Anastomotic leak occurred in three babies in the immediate postoperative period and all were successfully managed conservatively. Eight of the 11 patients developed anastomotic strictures; seven cases required 1 to 5 esophageal dilatations. One patient who did not respond to multiple esophageal dilatations required resection of an esophageal stricture. One patient died at 15 months of age of unrelated causes. The 10 surviving patients have been followed-up from 18 months to 11 years. At follow-up, seven patients were eating normally. Three patients had swallowing difficulties and all three were found to have esophageal strictures on barium swallow, two of them also had gross esophageal reflux and hiatus hernia. The height and weight in the 10 patients varied from 3rd centile to 75th centile. Delayed primary anastomosis is feasible in cases of isolated esophageal atresia and the patient's own esophagus is the best. A more aggressive approach should be applied to gastroesophageal reflux in these patients.

Management of pancreatic pseudocyst in childhood: an increasing role for percutaneous external drainage.

A total of four patients with a major pancreatic pseudocyst were treated by percutaneous pigtail catheter drainage. All patients recovered with complete resolution of the pseudocyst. There were no complications in relation to catheter insertion. Mean(s.d.) hospital stay was 56(15) days with a mean(s.d.) duration of catheter drainage and total parenteral nutrition of 21(14) and 35(9) days respectively. Percutaneous drainage is an appropriate method of first line therapy for non-resolving or enlarging pancreatic pseudocyst in children.

Primary hepatic tumours in children: a 26-year review.

Twenty-one children were admitted to a single paediatric institution between 1964-1990 with histologically proven primary liver tumours. The diagnosis was hepatoblastoma (HBL) in 15 patients, hepatocellular carcinoma (HCA) in 2, rhabdomyosarcoma (RMS) in 2, non-Hodgkin's lymphoma (NHL) in 1, and haemangioendothelioma (HE) in 1. The common presenting clinical features were anaemia, abdominal mass, and abdominal pain. Serum alpha-foetoprotein was useful in establishing a diagnosis in HBL and in monitoring disease activity. Computed tomographic (CT) scan, ultrasound, and angiography were useful preoperative investigations for assessing site and resectability of tumour. There were no survivors in patients with malignant hepatic tumours (n = 10) who had surgery alone prior to 1981. Of 7 patients with HBL diagnosed after 1981 who had adequate surgical resection and chemotherapy, 5(72%) are currently alive and disease free between 15 months and 8 years from diagnosis. We conclude that adequate surgical resection and adjuvant chemotherapy can improve disease free survival for children with HBL. Optimal treatment has yet to be devised for other malignant hepatic tumours.

Compensatory hypertrophy effectively assesses the degree of impaired renal function in unilateral renal disease.

A series of 66 children with unilateral renal disease and normal total renal function were reviewed with the aim of determining whether clinically detectable compensatory hypertrophy indicated severe contralateral renal impairment. The proportion of total renal function that was contributed by each kidney was determined using isotope renography (99mTc-DTPA for 46 children, 99mTc-DMSA for 20). Kidney length was measured from plain abdominal radiographs or intravenous urograms and hypertrophy was expressed as the renal size index (RSI). Of 37 kidneys with a RSI greater than 2 standard deviations from the mean, 36 had less than 15% of total function in the contralateral diseased kidney. Kidneys with less than 10 or 15% of total renal function could be selected with 97 or 80% sensitivity and 84 or 97% specificity, respectively, using the same RSI selection level.

Splenic pseudocyst: aspiration or cyst decapsulation.

A 12 year old boy with a splenic pseudocyst is reported. Percutaneous drainage resulted in complete emptying but rapid reaccumulation. Cyst decapsulation with splenic preservation was curative and is recommended for definitive therapy of splenic pseudocysts.