Wilms' tumor and horseshoe kidneys: a case report and review of the literature.

The presence of Wilms' tumor with a horseshoe kidney is an unusual combined clinical presentation. It has been reported that the incidence of Wilms' tumor in patients with horseshoe kidneys is higher than that seen in the general population. The current report describes a 5-year-old boy who presented with a stage III Wilms' tumor in a horseshoe kidney. The patient was treated with neoadjuvant chemotherapy followed by surgical resection, adjuvant chemotherapy, and radiation. The patient is disease free 40 months after diagnosis. A review of all reported cases of Wilms' tumor with horseshoe kidneys in the English-language literature before July 2002 is presented.

Injuries from falls in the pediatric population: an analysis of 729 cases.

BACKGROUND/PURPOSE: Falls are classified as low or high level for triage purposes. Because triage criteria dictate less urgency for low-level falls, this classification scheme has important implications for pediatric emergency care. METHODS: Retrospective analysis was conducted of 729 (393 low-level and 336 high-level) pediatric patients treated for fall-related trauma (1992 through 1998). Falls were classified as low (<15 feet) or high-level (> or =15 feet). All falls were reported as accidental or unintentional. RESULTS: The overall mortality rate was 1.6% (2.4% for high-level falls compared with 1.0% for low-level falls). All 4 patients who died of a low-level fall had an abnormal head computed tomography (CT) scan and intracranial hypertension. Half of deaths from high-level falls were attributable to intracranial injuries, and half were caused by severe extracranial injuries. Common extracranial injuries were upper extremity fracture (6.2%), lower extremity fracture (5.6%), pulmonary contusion (1.8%), pneumothorax (1.1%), liver laceration (1.1%), bowel injury (1.0%), and splenic injury (2.1%). Orthopedic and thoracic injuries resulted more commonly from high-level falls, whereas abdominal injuries were as likely to occur after a low-level fall. CONCLUSIONS: Intracranial injury accounts for the majority of deaths from falls. Children suffering low-level falls were at similar risk for intracranial and abdominal injuries compared with those who fell from greater heights. Pediatric trauma triage criteria should account for these findings.

Cardiac and great vessel injuries in children after blunt trauma: an institutional review.

PURPOSE: The purpose of this study was to review the incidence of cardiac and great vessel injury after blunt trauma in children. METHOD: A retrospective review of 2,744 patients with injuries from blunt mechanisms was performed. RESULTS: Eleven patients sustained cardiac injury. Four patients had clinically evident cardiac contusions. All recovered. Four patients who died from central nervous system injury were found to have cardiac contusions at autopsy. None had clinical evidence of contusion before demise. One patient had a traumatic ventricular septal defect (VSD) that required operative repair. Autopsy findings showed a VSD in another patient, and a third patient was found to have a ventricular septal aneurysm that was treated medically. Two patients had great vessel injuries. One patient had a contained disruption of the superior vena cava that was managed nonoperatively. Another patient had a midthoracic periaortic hematoma without intimal disruption found at autopsy. One patient had cardiac and great vessel injuries. Discrete aneurysms of 2 coronary artery branches and the pulmonary outflow tract were identified by cardiac catheterization. This patient was treated nonoperatively. CONCLUSIONS: Cardiac and great vessel injury after blunt trauma are uncommon in children. Cardiac contusion was the most common injury encountered but had minimal clinical significance. Noncontusion cardiac injury is rare. No patient with aortic transection was identified.

Coexistent neurenteric cyst and enterogenous cyst. Further support for a common embryologic error.

The authors describe common embryological pathways responsible for coexistent neurenteric and enterogenous cysts in a patient with spinal dysraphism.

Gastric duplication-colonic fistula with colonic ulceration and bleeding.

An 11-month-old girl had massive rectal bleeding. A midabdominal mass was palpated. Ultrasound scan showed a large cystic mass with diffusely echogenic contents; Tc-99M radionuclide scan confirmed a duplication. Exploratory surgery and pathology examination showed a gastric duplication with formation of a fistula into the transverse colon with a colonic ulcer and hemorrhage. This combination of findings is presented as a rare cause of lower gastrointestinal hemorrhage in infancy.

Dual patency of the omphalomesenteric duct and urachus.

An infant with umbilical cord discharge due to both a persistent urachus and an omphalomesenteric duct is described. The diagnosis was made preoperatively using fluoroscopy and MR imaging.

Hepatic hemangiomas in infants and children: a review of 30 cases.

From 1958 through 1992, 30 patients with hepatic hemangiomas were seen and treated at Children's Hospital Los Angeles. The majority of the patients were less 1 month of age (90 % younger than the first year of life) and there was no difference in sex distribution. Patients presented with coagulopathy, heart failure, abdominal mass, and respiratory distress. Eleven patients (33%) had hemangiomas in other sites. Fourteen patients were treated with steroid therapy. Of these, eight patients did not respond and received radiotherapy. Eleven patients who had the hemangioma confined to an anatomical lobe had resection of the hemangioma by liver lobectomy. Five of the most recent patients were successfully treated with hepatic artery embolization. Two other patients who were seen many years ago underwent diagnostic laparotomy and biopsy of the lesion before treatment with steroids. In one patient who presented with ruptured hepatic hemangioma, hepatic arterial ligation was performed. In another patient, seen recently, treatment with interferon alpha-2 was initiated, but the patient died. There were six deaths in the series. Four patients died of intractable congestive heart failure, steroids are given first. Course of steroid therapy may be repeated if necessary. Whereas formerly radiation therapy was added to the treatment of a patient resistant to steroids, therapeutic hepatic arterial embolization is a very good alternative for these patients. Surgical excision of the lesion can be performed by liver lobectomy if there is a solitary hemangioma within the boundaries of the surgical excision. Recently, in massive hemangioma with intractable thrombocytopenia, interferon alpha-2 therapy has been used, but so far our experience of this mode of therapy is limited.

Failure of technetium-99m hexamethylpropylene amine oxime leukocyte scintigraphy in the evaluation of children with suspected appendicitis.

To determine the accuracy and reliability of a labeled leukocyte imaging technique in the early diagnosis of pediatric appendicitis, we prospectively studied patients presenting to the pediatric emergency department with abdominal pain suggestive of appendicitis. Patients scheduled for urgent laparotomy were excluded, as were postmenarchal females. Blinded interpretations by two independent radiologists were compared with surgical findings, if managed operatively, or with discharge diagnoses and three week follow-up, if managed medically. Twenty-three children underwent technetium-99m hexamethylpropylene amine oxime (HMPAO) leukocyte scintigraphy. Seven had pathologically proven appendicitis, with false negative interpretations made in three and five cases by the two radiologists. Sixteen patients had prompt resolution of symptoms; however, scintigraphic abnormalities were identified in 10 and seven cases by the two radiologists. Resulting sensitivity, depending on the individual reader, ranged from 29 to 57%, with specificity 38 to 56%, positive predictive value 22 to 29%, negative predictive value 64 to 67%, and accuracy 43 to 48%. Interrater reliability for agreement on the scintigraphic diagnosis of appendicitis was poor (kappa = 0.38). Technetium-99m HMPAO leukocyte scintigraphy was neither accurate nor reliable as a diagnostic tool in a subgroup of pediatric patients with an initial clinical presentation equivocal for appendicitis. This finding contradicts previously published experience using similar scintigraphic techniques.

Duplications of the alimentary tract in infants and children.

Duplications of the alimentary tract are rare congenital anomalies that could present a diagnostic as well as therapeutic challenge. Twenty-seven patients with duplications of the alimentary tract were treated at Childrens Hospital Los Angeles between 1961 and 1992. Ages ranged from a few days to 5 years (67% younger than 1 year). The most common symptoms were nausea and vomiting, and the most common sign was a palpable abdominal mass. Three patients presented with gastric duplication, which was excised. The majority of the duplications were in the jejunum and ileum. All patients except one had primary resection of the duplication. One patient with a 45-cm tubular jejunal duplication was treated with mucosal stripping of the duplication. Five patients had cecal duplication, three patients presented with melena because of ectopic gastric tissue in the duplication, and two presented with intestinal obstruction. One of the latter patients presented with intussusception with cecal duplication as the leading point. Three patients with colonic duplication presented with abdominal pain and vomiting leading to excision of the duplication. Of the five patients with rectal duplication, three presented with chronic constipation. The other two patients presented elsewhere with perianal swelling, which eventually was drained because of a mistaken diagnosis of perianal abscess. Subsequently, these two patients came to us with persistent perineal fistula. In all our patients, rectal duplications were removed through a sacroperineal incision. The only patient in this series who died was a 6-week-old boy with gastric duplication; his death was attributed to an associated severe cardiac lesion.(ABSTRACT TRUNCATED AT 250 WORDS)

Hamartomas of the chest wall in infants.

Chest wall hamartomas in infancy are rare lesions with distinct clinical, radiologic, and pathologic characteristics. Four cases treated at Children's Hospital of Los Angeles are presented and previously reported cases are reviewed. Chest wall hamartomas arise antenatally and present as hard, immobile masses, which may cause respiratory insufficiency. An extrapleural mass arising from the ribs can be seen radiographically. Histologically, these lesions are hypercellular and consist of a disorganized array of mesenchymal tissues endogenous to the chest wall. Rapid growth may occur, but usually is self-limited. Chest wall hamartomas are usually benign. This series includes the malignant transformation of one of these lesions. En bloc resection is curative, but the large residual chest wall defect frequently results in scoliosis.

Efficacy of passive immunotherapy in experimental postsplenectomy sepsis due to Haemophilus influenzae type B.

In an effort to develop a more effective therapy for postsplenectomy sepsis, ceftriaxone and human intravenous immunoglobulin (IVIG), alone and in combination, were evaluated for their efficacy against experimental Haemophilus influenzae type B (Hib) bacteremia in splenectomized and sham-operated infant rats. Five-day-old animals had either a splenectomy or sham operation. At 12 days of age, they were challenged intraperitoneally with Hib. Fifteen hours later blood specimens were obtained for quantitative bacterial cultures, and immediately thereafter therapy was started with ceftriaxone, IVIG, combination of ceftriaxone and IVIG, or albumin (control). Quantitative blood cultures were repeated 24 and 48 hours after the treatment. Prior to the treatments, splenectomized animals had significantly higher bacterial counts in blood when compared with sham-operated animals (P < .001). Splenectomized animals receiving IVIG, ceftriaxone, or the combination of IVIG and ceftriaxone had significantly increased bacterial clearance from blood when compared with the controls (P < .01). In addition, animals treated with ceftriaxone or the combination of IVIG and ceftriaxone had significantly increased bacterial clearance compared with the IVIG alone treatment group (P < .01). Overall, the mortality was significantly higher in splenectomized animals compared with the sham-operated animals (P < .05). The control animals had significantly higher mortality compared with the IVIG, ceftriaxone, and combined ceftriaxone and IVIG treatment groups (P < .05). There were no detrimental effects of combining IVIG and ceftriaxone together.

Megacystis microcolon intestinal hypoperistalsis syndrome: late sequelae and possible pathogenesis.

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is characterized by hypoperistalsis in the presence of ganglion cells, malrotation, microcolon, bladder distension, and female predilection. We draw attention to the long-term management of a patient with MMIHS and propose a pathogenetic mechanism to account for this syndrome. We propose that the initial event in the pathogenesis of MMIHS is an intramural inflammatory process that affects the gastrointestinal and urinary tracts. This leads to extensive fibrosis which destroys the intestinal neural network, producing hypoperistalsis. The same process causes neuromuscular incoordination in the bladder wall, resulting in irregular bladder contractions against a "closed sphincter" leading to bladder distension. The enlarged bladder then interferes with the rotation of the intestine causing malrotation.

Neuroblastoma masquerading as a retroperitoneal Salmonella abscess.

Symptomatic Salmonella infections usually manifest as self-limited gastrointestinal distress. Patients with chronic systemic illnesses or those who are immunosuppressed may rarely present with Salmonella infection as distant suppurative abscesses. We present a previously healthy Armenian boy who came to medical attention with abdominal pain, fever, and anemia. Abdominal computed tomography (CT) scan showed a cystic suprarenal mass that was surgically explored and found to be a retroperitoneal Salmonella abscess. Postoperative CT scan showed resolving inflammation. A 6-month follow-up CT showed a large suprarenal tumor, which at exploration was found to be neuroblastoma. To our knowledge, Salmonella has never been reported presenting as a solitary retroperitoneal abscess, and neuroblastoma has not been described presenting as a Salmonella abscess. The patient is also unusual because the abscess contained a species unusual for suppurative salmonellosis.

Treatment of bilateral Wilms' tumor: comparison of initial biopsy and chemotherapy to initial surgical resection in the preservation of renal mass and function.

The National Wilms' Tumor Study 3 (NWTS-3) recommended treatment of bilateral Wilms' tumor with initial biopsy followed by chemotherapy with subsequent operation to resect the remaining tumor. This study was performed to determine if this approach preserves renal mass and function when compared with initial surgical resection followed by chemotherapy. Over a 20-year period (1970 to 1990), 15 patients with synchronous bilateral Wilms' tumor were treated at the Childrens Hospital of Los Angeles. Eight patients in the surgical group underwent initial unilateral nephrectomy with contralateral biopsy, wedge resection, or partial nephrectomy and subsequent chemotherapy. The seven patients in the chemotherapy group underwent bilateral tumor biopsy, followed by chemotherapy and subsequent tumor resection. Patients were assigned to each group in a nonrandomized manner according to the preference of the attending oncologist and surgeon. Comparison of the two groups showed no significant differences in sex distribution, initial renal function, tumor histology, dose and field of radiotherapy, presence or absence of positive surgical margins, and local recurrence rates. Patients in the surgery group were slightly older than those in the chemotherapy group: 3.6 +/- 2.2 versus 2.3 +/- 2.2 years. The percent of renal mass involved by tumor for the surgery group was 52 +/- 12 versus 73 +/- 16 for the chemotherapy group (P = .03). The percent of renal mass preserved following all operations, local recurrence rates, incidence of metastases, and survival was nearly identical between the two groups. There were three cases of renal failure in the surgery group and one case of renal failure in the chemotherapy group.(ABSTRACT TRUNCATED AT 250 WORDS)

Communicating bronchopulmonary foregut malformations: classification and embryogenesis.

Communicating bronchopulmonary foregut malformations (CBPFMs) are characterized by a fistula between an isolated portion of respiratory tissue (ie, a lung, a lung lobe, or a segment) and esophagus or stomach. We combine our 30-year (1959 to 1989) experience of 6 cases with 51 reported patients to propose a CBPFM classification supported by a proposed embryogenesis theory. Group I (16%): anomaly is associated with esophageal atresia and tracheoesophageal fistula. Group II (33%): one lung originates from the lower esophagus. Group III (46%): an isolated anatomic lung lobe or segment communicates with the esophagus or stomach. Group IV (5%): A portion of the normal bronchial system communicates with the esophagus. The portion of the lung served by the communicating bronchus receives systemic blood supply. The right and left lung sacs curve dorsally to embrace the lower esophagus during normal lung development. At this stage a part of the lung bud joins the esophagus. This segment then breaks away from the main pulmonary anlage to form a CBPFM. CBPFMs should be considered in the workup of infants with respiratory distress and/or recurrent pneumonias. Patients with suspected pulmonary sequestration should undergo contrast studies to exclude a gastrointestinal communication.

Partial splenic embolization. An effective alternative to splenectomy for hypersplenism.

Splenectomy for massive splenomegaly and hypersplenism carries a significant morbidity and mortality. We have used partial splenic embolization (PSE) as an effective alternative to splenectomy. Ten PSE procedures were performed on nine patients without mortality and with minimal morbidity. The age of the patients ranged from 8 months to 32 years (mean 14 years). The causes of splenomegaly and hypersplenism included cystic fibrosis with cirrhosis (2), tyrosinemia and cirrhosis (1); thalassemia (1), hemophilia with Human Immune Deficiency Virus infection (2), chronic hepatitis with portal hypertension (1), malignant histiocytosis (1), and Wiskott-Aldrich Syndrome (1). All procedures were performed under local anesthesia with sedation. A percutaneous femoral artery approach to the splenic artery was used to deliver Ivalon sponge particles (280-800 microns) into the spleen. Splenic infarction was assessed by postembolization angiograms. All of the patients except one demonstrated improvement of hematologic parameters. In one patient, however, cytopenia improved only after a second embolization. In the total series, there was an early mean rise of 8,600/mm3 in the leukocyte count (range 2,900-14,900) and 212,000/mm3 in the platelet count (range 30,000-718,000). Follow-up ranged from 4 months to 7 years. Improvement of the blood picture has been persistent in seven of the eight patients who showed initial improvement. Transient procedural complications included fever (5), pleural effusion (2), pneumonia (1), and splenic abscess (1). One patient had paralytic ileus lasting for 10 days and one patient developed a streptococcal peritonitis 3 weeks after embolization. No patient developed pancreatitis or vascular compromise of other abdominal viscera.(ABSTRACT TRUNCATED AT 250 WORDS)

Emergency center laboratory evaluation of pediatric trauma victims.

Emergency center (ER) trauma evaluations often include leukocyte count (LC), serum amylase (SA), electrolytes (EL), and urine analysis. We reviewed records of 100 pediatric ER patients to determine utility of these tests in management of blunt injury. SA was evaluated in 65 patients and ranged from 30-146 U/L (mean 50.6 U/L); 14 patients with normal CT scans had SA from 30-68 U/L (mean 49.1 U/L). Six patients with intraabdominal or retroperitoneal injuries had SA from 30-130 U/L (mean 64.0 U/L), P = NS. LC was determined in 76 patients and ranged 2.3-28.3 k/ml (mean 13.8 k/ml). Patients with normal abdominal CT (12) had mean LC 14.8 k/ml (range 7.2-19.6 k/ml). Eight patients with injuries on CT had mean LC 14.4 k/ml (range 3.5-27.1 k/ml). ER, SA, and LC did not alter patient management. Thirty-four patients had serum sodium, 36 potassium, and 33 chloride and bicarbonate determinations. Sodium, potassium, and chloride levels were uniformly normal; bicarbonate and leukocyte counts were uniformly abnormal in initial evaluations. These changes are expected in response to severe injury and their determinations did not alter patient care. Combined laboratory urinarlysis (LA) and urine dipstick (DA) analysis for hematuria had sensitivity 75.0 per cent (specificity 81.6%). LA predicted injury with sensitivity 75.0 per cent (specificity 81.6%). DA predicted injury with sensitivity 60.0 per cent (specificity 79.2%). DA accurately represented LA results (sensitivity 100%, specificity of 94.5%). DA is a rapid and effective replacement of LA in evaluation of trauma patients in the emergency center.

Surgical implications of Henoch-Schönlein purpura.

Intestinal intussusception, perforation, necrosis, and massive gastrointestinal bleeding are the surgical conditions most often seen in Henoch-Schönlein purpura (HSP). We reviewed the charts of 183 patients who were diagnosed with HSP at two children's hospitals over a 17-year period. Ten patients underwent laparotomy. Intussusception was the most common surgical lesion. Sequential barium enema and upper gastrointestinal series established the diagnosis of intussusception preoperatively in five of six patients. Two patients developed jejunal perforation and segmental ileal ischemia. Although gastrointestinal bleeding occurred in 52% of patients, the bleeding was self-limiting and no blood transfusion was required. Laboratory data were not helpful in differentiating patients with and without surgical problems.

Pseudocysts of the pancreas in children.

Sixteen children with pancreatic pseudocysts were treated from 1965-1988. Blunt trauma was the etiology of pseudocyst formation in 69 per cent of children with 50 per cent resulting from the abdomen impacting bicycle handlebars. Chronic pancreatitis is an uncommon cause of pseudocyst formation in children. Medical therapy is directed towards reduction of pancreatic stimulation and nutritional support, which are maintained through pseudocyst resolution or maturation. Pseudocysts spontaneously resolved in 25 per cent of patients. Complications occurred in 25 per cent during nonoperative management. Children may safely undergo internal drainage earlier than adults (3-4 weeks vs 6 weeks). Internal drainage by cystoenterostomy was curative in eight patients. Persistent fistula drainage developed for five weeks in one patient who had surgical external pseudocyst drainage. One patient required distal pancreatectomy for a transected pancreatic duct. Spontaneous resolution of psseudocysts while on medical therapy is more frequent in children than in adults, and major complications (abscess formation, hemorrhage, and fistula formation) are usually not encountered. Pseudocyst rupture is the major complication of conservative management. We had no pseudocyst recurrences and 11 of 12 children treated surgically were discharged home within ten days of operation.