James J. Murphy

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 frequency of apneas in premature infants after inguinal hernia repair: do they need overnight monitoring in the intensive care unit?

BACKGROUND Postoperative apneas are reported in up to 49% of premature infants undergoing anesthesia for inguinal hernia repair. Our current practice is to monitor all of these babies in the intensive care unit (ICU) overnight after surgery. In addition to the considerable expense to the health care system, these cases are cancelled if no ICU bed is available. METHODS A retrospective chart review of all premature infants undergoing inguinal hernia repairs over the past 5 years was undertaken. All postoperative apneas were identified. Potential risk factors were evaluated. RESULTS Five (4.7%) of 126 premature infants had apneas after inguinal hernia repair. All of these babies had a previous history of apneas. They also had lower weights both at birth (1.08 vs 1.73 kg) and at the time of surgery (3.37 vs 4.4 kg) as well as lower gestational ages (29 vs 32.3 weeks). They were much more likely to have a complicated past medical history. Markers for this included intraventricular hemorrhage, patent ductus arteriosus, bronchopulmonary dysplasia, and requirement for mechanical ventilation and supplemental oxygen after birth. The use of sevoflurane was the only anesthetic factor which had significance. CONCLUSION Postoperative apnea in premature infants after inguinal hernia repair using current anesthetic techniques is much less common than previously reported. Infants with prior history of apneas are at highest risk. Other risk factors appear to include gestational age, birth weight, weight at time of surgery, and a complicated neonatal course. Selective use of postoperative ICU monitoring for high-risk patients could result in significant resource and cost savings to the health care system.

Morbidity in neurologically impaired children after percutaneous endoscopic versus Stamm gastrostomy.

Neurologically impaired children frequently require a feeding gastrostomy. Few reports are available comparing the incidence of postoperative complications and symptomatic gastroesophageal reflux after endoscopic versus operative Stamm gastrostomy in this group of children. We undertook a retrospective study of 63 consecutive neurologically impaired children requiring a feeding gastrostomy, with an average of 23 months of follow-up. No child had symptomatic gastroesophageal reflux. Thirty children had a percutaneous endoscopic gastrostomy and 33 had a Stamm gastrostomy, depending on the preference of the surgeon. The two groups were comparable in age range, cause of neurologic impairment, and indication for gastrostomy. Minor complications occurred in 30%. All three major complications occurred after Stamm gastrostomy, including two postoperative deaths. Symptomatic gastroesophageal reflux developed in 60%. The incidence of fundoplication after gastrostomy was 10% in the percutaneous endoscopic gastrostomy group and 39% after Stamm gastrostomy (p < .025). Morbidity was lower after percutaneous endoscopic gastrostomy than after Stamm gastrostomy in this group of neurologically impaired children. Fundoplication for symptomatic gastroesophageal reflux was infrequent after percutaneous endoscopic gastrostomy and significantly more common after Stamm gastrostomy. Percutaneous endoscopic gastrostomy is recommended as the initial procedure in neurologically impaired children without symptomatic gastroesophageal reflux who require a feeding gastrostomy.

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.

Traumatic pseudoaneurysms of the liver and spleen in children: is routine screening warranted?

BACKGROUND Although blunt injury to the spleen and liver can lead to pseudoaneurysm formation, current surgical guidelines do not recommend follow-up imaging. Controversy exists regarding the clinical implications of these traumatic pseudoaneurysms as well as their management. METHODS Retrospective review of children treated nonoperatively for isolated blunt liver and spleen trauma between 1991 and 2008 was undertaken. Patient demographics, grade of injury, and follow-up Doppler ultrasound results were obtained. RESULTS Three hundred sixty-two children were identified. One hundred eighty-six of them had splenic injuries, and 10 (5.4%) developed pseudoaneurysms. They were associated with grade III (3/39 [8%]) and grade IV (7/41 [17%]) injuries. In 7 patients, the pseudoaneurysm thrombosed spontaneously. Angiographic embolization was required in 2 children, and one underwent emergency splenectomy for delayed hemorrhage. Of the 176 patients who had liver injuries, 3 (1.7%) developed pseudoaneurysms. All 3 were associated with grade IV injuries (3/11 [27%]). One child underwent early embolization, while 2 developed delayed hemorrhage requiring emergent treatment. CONCLUSIONS Pseudoaneurysm development after blunt abdominal trauma is associated with high-grade splenic and liver injuries. Routine screening of this group of patients before discharge from hospital may be warranted because of the potential risk of life-threatening hemorrhage.

Fibromatoses: Clinical and Pathological Features Suggestive of Recurrence

BACKGROUND/PURPOSE Fibromatoses represent a spectrum of nonneoplastic spindle cell tumors frequently identified in childhood. Although they may be locally aggressive, they do not metastasize. The authors have reviewed their experience of these lesions in an attempt to identify clinical and pathological features suggestive of recurrence. METHODS Clinical records and pathological specimens from 55 cases of fibromatosis diagnosed and treated at British Columbias Childrens Hospital from 1982 to 1995 were reviewed. RESULTS Thirty boys and 25 girls with ages ranging from 1 month to 14 years were identified. Eighteen tumors (33%) were congenital. Diagnoses were as follows: musculoaponeurotic fibromatosis (n = 27), infantile myofibromatosis (n = 10), Dupuytren-type fibromatosis (n = 7), fibromatosis colli (n = 7), and digital fibroma (n = 4). Clinical follow-up showed a survival rate of 98%. The single mortality occurred in an infant born with an intraabdominal visceral myofibromatosis, which proved unresectable. Fifteen cases recurred (27%), and nine cases (16%) of infantile myofibromatosis spontaneously regressed. All remaining cases were controlled successfully by surgical resection. CONCLUSIONS Clinical features suggestive of recurrence included (1) presentation at greater than 5 years of age, (2) extremity location of disease, and (3) incomplete surgical resection. Pathological features suggestive of recurrence were (1) microscopic evidence of tumor at resection margins, (2) mitotic index of 5 or more per 10 high-power fields, and (3) areas of necrosis and inflammation within the tumor.

Multiple entero-entero fistulae : An unusual complication of Henoch-Schönlein purpura

Henoch-Schönlein purpura (HSP) is an immunologically mediated systemic vasculitis of small blood vessels that frequently involves the gastrointestinal tract. Surgeons often are asked to assess patients with HSP for abdominal pain. Common complications that lead to surgical intervention include intussusception, perforation, necrosis, and massive gastrointestinal bleeding. However, the development of multiple entero-entero fistulae has not been described previously. Herein the authors describe the case of a 10-year-old girl with HSP who had a typical rash, seizures secondary to central nervous vasculitis, and gastrointestinal involvement. The abdominal pain persisted for several days until marked fever and hypotension developed, which necessitated surgical intervention. During laparotomy, multiple entero-entero fistulae of the ileum were found, which required resection and primary anastomosis. Histological examination of the ileum was consistent with HSP vasculitis. The patient made an unremarkable recovery and has had no recurrence of symptoms.

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.