Retained common-duct stones after open cholecystectomy and duct exploration in children.

A retrospective study was performed of 250 patients with cholelithiasis treated at the Royal Children's Hospital, (RCH) Melbourne, over 25 years by open operation; 32 (12.8%) had proven choledocholithiasis on either preoperative imaging, operative cholangiography (OpCG), or postoperative investigation. A further 3 had underlying congenital biliary abnormalities and were excluded from further study. Thirty-one of the 32 were explored at open operation, 27 after OpCG and 4 on clinical grounds. One retained common-bile-duct (CBD) stone was undetected until the postoperative period (1/250, 0.25%). Seven ducts were not cleared, giving a duct exploration failure rate of 22.6% (7/31). All 8 retained CBD stones were identified in the early postoperative period and managed with a variety of techniques, including endoscopic retrograde cholangio-pancreatography (ERCP). The incidence of retained stones after open CBD exploration was high (22.6%), and can be attributed to difficulties in operative technique dealing with the smaller paediatric CBD. In addition, haemolytic disease seems to induce a propensity for choledocholithiasis. Given the small numbers presenting with cholelithiasis to RCH (10 per year), it is suggested that a selective approach to CBD exploration is appropriate in children. With the increasing use of laparoscopic cholecystectomy in children and the inherent technical difficulties of laparoscopic operative cholangiography, ERCP may offer an alternative solution in dealing with CBD stones rather than open or laparoscopic CBD exploration.

Review of the outcome of management of Graves' disease in children and adolescents.

OBJECTIVE: To review our recent experience in the management of paediatric Graves' disease, the leading cause of hyperthyroidism in childhood, in view of ongoing international debate regarding therapy. METHODOLOGY: Retrospective medical record review of patients with Graves' disease, treated between 1985 and 1999, at the Royal Children's Hospital, Melbourne. RESULTS: Forty patients, aged at diagnosis between 2.9 and 17.6 years (median age 11 years; 31 females), were treated. Thirty-three patients received either definitive therapy or at least 24 months medical therapy and follow up at the time of the study. Six patients experienced successful remission upon withdrawal of medication (median medication duration 3.2 years; four females; median follow up 4.1 years). Children successfully achieving remission were younger at presentation than those requiring surgery (9.5 vs 12.9 years, respectively; P = 0.01). Ten patients were treated surgically, leaving 4-6 g residual thyroid tissue. Surgical indications included failed initial medical therapy, relapse after medication cessation, non-compliance and significant medication side effects. During a median follow up of 1.7 years (range 0.2-5 years), three of 10 patients have subsequently relapsed and four of 10 patients are receiving postoperative thyroxine replacement. Transient hypocalcaemia occurred in five patients and temporary recurrent laryngeal nerve palsy occurred in two patients. CONCLUSIONS: Fewer than 20% of children experienced successful sustained remission at median follow up of 3.2 years. Medication remains a well-tolerated therapeutic option. Surgery is a reasonable alternative when there are specific indications. In order to decrease the likelihood of surgical relapse, less than 4 g thyroid tissue should be left postoperatively.

Ovarian torsion in inguinal hernias.

The possibility of sustaining ovarian damage as a consequence of an inguinal hernia becoming strangulated is well recognised. However, the mechanism by which this gonadal damage occurs is rarely stated; it is assumed that the injury is secondary to compression of ovarian vessels by an entrapped bowel loop. Eleven cases of ovarian torsion within an inguinal hernial sac are reported. In all cases the primary aetiological event was torsion of the ovary and tube on its pedicle whilst suspended from the neck of the hernial sac. This mechanism constituted the major cause of ovarian damage within irreducible herniae in our series.

Tension pneumothorax secondary to grass head aspiration.

Aspiration of inflorescence or grass heads (seed head of grasses) often presents with atypical signs and symptoms because grass heads have a tendency to rapidly migrate to the periphery of the lung. If this is not recognized, it can lead to delay in diagnosis and serious complications. Removal with rigid bronchoscopy maybe difficult, and surgery is often needed. We report a case of a seven-month-old child who had a delayed diagnosis of grass head aspiration and subsequently presented with a life threatening tension pneumothorax. This case highlights the importance of obtaining a detailed history in cases of foreign body aspiration and the need to include it in the differential diagnosis of unexplained respiratory symptoms, especially those of sudden onset in children.

Primary cutaneous Ewing's sarcoma/peripheral primitive neuroectodermal tumors in childhood. A molecular, cytogenetic, and immunohistochemical study.

Childhood cutaneous and subcutaneous malignancies are rare and include metastatic tumors of diverse histogenesis as well as primary lesions, such as sweat gland carcinomas. Some cutaneous malignancies exhibit a small round cell tumor morphology with few definitive differentiating features; they can thus pose a significant diagnostic problem. We describe two primary malignancies of the skin and superficial subcutis, which were originally diagnosed as sweat gland carcinomas on the basis of their morphological features. A cytogenetic analysis performed on one of these lesions showed the t(11;22)(q24;q12) rearrangement, believed to be unique to the Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) group of neoplasms. In view of this unexpected result, reverse transcriptase-polymerase chain reaction analysis was performed on both lesions and showed that they expressed EWS/FLI-1 fusion gene mRNA transcripts, the molecular equivalent of t(11;22)(q24;q12). The two tumors also had an immunohistochemical profile suggesting ES/pPNET, including strong expression of the MIC2 antigen. Both patients were treated with wide local excision, and one was given a course of chemotherapy. Neither patient showed evidence of tumor elsewhere after follow-up periods of 2 years and 16 years. These findings suggest that these tumors are indeed a form of primary ES/pPNET arising in the skin or superficial subcutis, which may be of low-grade malignancy and curable by local surgery.

Trends in the use of fundoplication in children with gastro-oesophageal reflux.

OBJECTIVE: To document changing trends in the indications for fundoplication in children with gastro-oesophageal reflux and to identify the main shortcomings and complications of the procedure. METHODOLOGY: Retrospective review of 405 consecutive children undergoing fundoplication between 1978 and 1992 inclusive. RESULTS: Trends in the indications for fundoplication related to broadening of its use in those with severe neurological impairment and uncontrolled reflux, and to changes in the management of patients in the neonatal period. Complications included unwrapping with recurrence of reflux, hiatal hernia, adhesive small bowel obstruction, dumping syndrome, post-operative persistence of oesophageal stricture, excessively tight wrap and poor oesophageal clearance (mostly in oesophageal atresia patients). CONCLUSIONS: This study identified those patients most likely to develop complications following fundoplication. This may assist in the pre-operative assessment of the likely benefit of fundoplication in children who often have other complex problems.

Management of the premature infant with esophageal atresia and hyaline membrane disease.

Prematurity of 34 weeks' gestation or less occurs in 12% of infants born with esophageal atresia and distal tracheoesophageal fistula, and is likely to result in moderate or severe hyaline membrane disease. In the past, these infants have often had their surgical treatment delayed or staged. Based on our experience of 24 such patients since 1977, we propose that optimal treatment involves early division of the distal tracheoesophageal fistula via a thoracotomy and simultaneous repair of the esophagus where the patient's condition permits. This should be in the first 12 hours of life, before the hyaline membrane disease becomes fully established. Gastrostomy is not required and may be contraindicated.

Secondary esophageal surgery following repair of esophageal atresia with distal tracheoesophageal fistula.

During the period 1948 through 1988, 498 patients with esophageal atresia and distal tracheoesophageal fistula were admitted to the Royal Children's Hospital, Melbourne. Fifty patients had a second operative procedure on the esophagus, for anastomotic stricture (30), recurrent fistula (15), both (4), and a postmyotomy diverticulum (1). During the same period, nine patients underwent esophageal replacement and 33 patients a Nissen fundoplication. Improvements in the technique of esophageal anastomosis, and in recent years the use of fundoplication to correct gastroesophageal reflux have led to a marked reduction in the need for secondary surgery to the esophagus after repair of esophageal atresia. Esophageal replacement is rarely required in esophageal atresia and distal tracheoesophageal fistula. One-layer end-to-end esophageal anastomosis using interrupted sutures resulted in the lowest rate of recurrent fistula and anastomotic stricture.

Priapism in childhood.

Priapism is a rare condition in childhood; the majority of reported cases are in boys with sickle cell disease, where conservative management is often appropriate. Our experience with 4 cases, with differing aetiologies, and a review of the literature highlight the need for aggressive therapy when conservative measures fail.

Current surgical management of oesophageal atresia and/or tracheo-oesophageal fistula.

During the last 40 years, 579 babies with oesophageal atresia and/or tracheo-oesophageal fistula have been admitted to the Royal Children's Hospital (RCH), Melbourne. There are 393 survivors. Advances in the surgical management have contributed to lowering the morbidity and mortality rates. This paper describes current surgical management of each anatomical variant at RCH; specific reference is made to the problems of the long gap oesophageal atresia and prematurity.

Developments in the management of oesophageal atresia and tracheo-oesophageal fistulas.

Since 1948, when the first patient with oesophageal atresia and a tracheo-oesophageal fistula was treated successfully at the Royal Children's Hospital, Melbourne, 569 infants with one or both conditions have been managed at that institution. The mortality rate in those in whom surgical repair of the oesophageal atresia and distal tracheo-oesophageal fistula was attempted has declined from 55% in the first 10 years to less than 1% in the last 10 years of the series. Earlier diagnosis and improvements in resuscitation, transport, neonatal intensive care, anaesthesia, the treatment of associated anomalies and surgical technique all are likely to have contributed to the decline in the mortality and morbidity rates. Problems which remain unresolved in part relate to the aetiology and embryogenesis of oesophageal atresia, the management of long-gap atresia, and the treatment of gastro-oesophageal reflux and tracheomalacia. This article outlines the improvements in management which have occurred already and speculates about what the future may hold.

Intussusception: clinical prediction of outcome of barium reduction.

Patients with intussusception who have necrotic bowel requiring bowel resection or who are at major risk of perforation from attempted hydrostatic reduction may be better managed without barium reduction. Ideally, the clinician would like to identify such patients at presentation. Two groups of patients, representing the extreme ends of the intussusception treatment spectrum were investigated in order to highlight the clinical features of patients in whom an attempted barium reduction is not justified. A group of 200 patients who had successful and safe hydrostatic reduction was compared with 104 patients who ultimately required bowel resection. Four features were found to be indicators of an increased likelihood of resection: age 3 months or less, or greater than 2 years, duration of symptoms greater than 24 h, presence of small bowel obstruction on plain radiology, and clinical assessment of dehydration greater than 5%. The validity of these features as prognostic indicators was assessed by applying them to all patients who had attempted barium reduction to see how they predicted patient outcome. In isolation, each feature was found to be compatible with a safe and successful hydrostatic reduction. The rate of resection was increased in patients with multiple adverse features and in these patients the enema technique may require modification. Patients with three or four adverse features had an unacceptably high incidence of gangrenous bowel requiring resection and a low likelihood of successful hydrostatic reduction. It is believed that attempts at reduction are not appropriate in these patients.

Precocious puberty associated with malignant thoracic teratoma and malignant histiocytosis in a child with Klinefelter's syndrome.

A 10-year-old boy with precocious puberty of 2 1/2 years' duration presented with a malignant thoracic teratoma with elevated levels of beta human chorionic gonadotropin (beta-HCG) and alpha fetoprotein (alpha FP). The mediastinal tumor was completely excised and adjuvant chemotherapy commenced. Within 3 weeks of commencing chemotherapy he developed hematologic abnormalities that, with the subsequent clinical illness, led to a diagnosis of malignant histiocytosis. Death occurred a few days after start of therapy for malignant histiocytosis. Cytogenic studies showed the somatic karyotype to be that of Klinefelter's syndrome, while the malignant cells in marrow and lymph node carried an additional marker chromosome. This case, with others reported, suggest that the XXY karyotype may influence the development of mediastinal germ cell tumors, which in themselves appear to be associated with the early onset of a malignancy of the hemopoietic system.

Recurrent intussusception: barium or surgery?

A review was conducted of children with intussusception admitted to the Royal Children's Hospital over a 16 year period: of 630 episodes of intussusception, 28 represented recurrences (4.4%): 10 of these occurred in children aged 2 years or over. Duration of symptoms and the incidence of an incorrect initial diagnosis were markedly reduced with recurrence. The clinical presentation was similar to the first episode apart from rectal bleeding which was less common, reflecting the shorter history. Sixteen barium enemas were performed, of which 10 were successful. There were 18 operations with eight resections; seven for localized lesions and one for failure of manual reduction in a patient with an inverted Meckel's diverticulum. Barium reduction for recurrence should not be attempted in children over 2 years of age in whom no laparotomy was performed for their first episode, for most second recurrences, or in multiple polyposis.

Choledochal cyst presenting as acute pancreatitis.

A case of a 2 year old boy who presented with pancreatitis in association with choledochal cyst is reported. The pathogenesis, clinical presentation and surgical management are discussed.

Gallstones and duodenal atresia.

Two children with cholelithiasis several years after relief of a duodenal atresia are reported. A survey of the literature on duodenal atresia suggests that there may be associated abnormalities in the biliary tract in this condition, and raises the possibility of cholelithiasis developing in these patients many years after successful relief of the duodenal obstruction by some form of by-pass procedure.