G.C. Fraser

Rhabdomyosarcoma arising within congenital pulmonary cysts : report of three cases

Over the past 9 months, three cases of primary pulmonary rhabdomyosarcoma have been treated at British Columbia Childrens Hospital. Two patients (aged 24 and 37 months) presented with spontaneous pneumothoraces and had cystic changes in the affected lung on chest radiograph. The third patient (aged 42 months) was evaluated for chronic cough, fever, and failure to thrive. Chest x-ray showed a large mass in the left lower lobe as well as mediastinal adenopathy. All three of these lesions originated within congenital lung cysts, one a peripheral bronchogenic cyst and the others cystic adenomatoid malformations. This report suggests that there is a significant risk for the development of rhabdomyosarcoma within malformed pulmonary tissue.

Splanchnic artery pseudo-aneurysms secondary to blunt abdominal trauma in children

Traumatic splanchnic artery pseudo-aneurysms are uncommon; only two cases have been reported among the pediatric population. The authors describe their experience with four patients in whom splanchnic artery pseudoaneurysms developed after blunt abdominal trauma. Splenic artery pseudo-aneurysms were found in a 6-year-old boy and an 8-year-old girl after blunt splenic injuries. In both cases, spontaneous thrombosis of the pseudo-aneurysms occurred after a period of observation. Hepatic artery pseudoaneurysms were found in a 7-year-old boy and a 10-year-old girl after major liver lacerations. The boy had successful angiographic embolization of the lesion, but the girl required direct ligation of the pseudo-aneurysm after nearly exsanguinating from acute hemorrhage. All four children have recovered completely, with no long-term sequelae. Traumatic splanchnic artery pseudo-aneurysms are potentially life-threatening complications that can occur after blunt abdominal trauma. The investigation and management of these lesions must be individualized according to the clinical scenario.

The Bowel Management Tube: An Effective Means for Controlling Fecal Incontinence

Regular bowel washout enemas have been used as a method of management of fecal incontinence. The effective administration of a washout enema to a child with weak anal sphincters is often a problem. Using a new silastic balloon-tipped enema catheter (bowel management tube [BMT]) of our design, we prospectively studied its effectiveness in a group of children who suffered fecal incontinence. Thirty-one children were studied over a 1-year period. Their diagnoses included meningomyelocele (19), postoperative Hirschsprungs disease or imperforate anus (10), and other (2). Before and after starting the BMT enema system, clinical assessment and a diary, which graded the degree of fecal incontinence and satisfaction with the system, were completed. Five patients failed to benefit because of noncompliance (3) or balloon extrusion (2). Three more patients discontinued the use of the tube system. Twenty-three patients achieved successful results with this system as evidenced by a significant amelioration in their fecal incontinence and their unwillingness to give up the use of the BMT. We conclude that the use of a regular washout enemas with BMT can be an effective method for control of fecal incontinence in children.

Coagulopathy associated with large sacrococcygeal teratomas

Twelve neonates with sacrococcygeal teratoma (SCT) have been treated at British Columbia Childrens Hospital over the past 5 years. Clinically significant coagulopathy developed in four of these neonates and two died, one before surgical intervention could be undertaken. Disseminated intravascular coagulation (DIC) was found in one patient and thrombocytopenia in another on preoperative laboratory studies. Etiology of the coagulopathy is unclear, but appears to be multifactorial. Although several clinical reviews have noted mortalities due to exsanguinating hemorrhage, no study has focused solely on this issue. The diagnosis of SCT in the neonate at high risk for development of coagulopathy is usually made prenatally. Premature labor is often precipitated by associated polyhydramnios and large tumor size. Fetal distress, prematurity, and low birth weight are common. Presence of placentamegaly, hydrops fetalis, and congestive heart failure are ominous prognostic signs. Early identification of patients at increased risk for development of hemorrhagic complications may allow optimization of their management. Cesarean section should minimize trauma to the SCT during delivery. Expeditious resection of the lesion may improve survival.

The treatment of simultaneous choriocarcinoma in mother and baby

Simultaneous occurrence of choriocarcinoma in mother and child is rare. Such a case is described in which a massive intrahepatic tumor in the infant led to its treatment and that of the mother who showed evidence of the same tumor. Both are alive and well 1 year after treatment, the longest recorded infant survival.

Obstructive jaundice caused by neuroblastoma managed with temporary cholecystostomy tube

Neuroblastoma presenting as obstructive jaundice is very rare. The authors present two cases of neuroblastoma, one primary and one recurrent, manifesting as a malignant obstruction of the extrahepatic biliary system. Various methods of biliary decompression were considered in these children including transhepatic or retrograde biliary stenting and internal cholecystoenteric bypass. An attempt at percutaneous transhepatic stent placement failed in one case. In each patient, a simple insertion of a cholecystostomy tube proved effective. Immediately postoperatively, both patients had rapid resolution in symptoms and a decrease in bilirubin levels. Transient mild cholangitis in both children was successfully treated with antibiotics. Chemotherapy reduced the tumor size in each case, and the cholecystostomy tubes were removed within 3 weeks, after cholangiography showed patency of the distal common bile ducts. Temporary cholecystostomy tube drainage and systemic chemotherapy proved to be a safe, simple, and effective method for managing obstructive jaundice caused by neuroblastoma in these two cases.

‘Pilonidal appendicitis’ or ‘the hair of the dog’: An unusual case of foreign body perforation of the appendix

Perforation is common in the young preschool child who has appendicitis, and can occur as the result of an ingested foreign body. A variety of objects have been incriminated. The present report describes a case in which the appendiceal wall was pierced by a stiff canine hair.