Management of Wilms' tumor with intracardiac extension

 We review our experience and the literature in treating 4 patients with Wilms' tumor (WT) with intracardiac extension among 92 patients with this neoplasm. Cardiopulmonary bypass with circulatory arrest and profound hypothermia was used. There were 3 boys (3 years, 4 years 5 months, and 15 years) and 1 girl (6 years). The follow-up periods were 8 months, 3 years, 2 years 6 months, and 15 years, respectively. We had no surgical complications and conclude that the preoperative diagnosis is extremely important. These patients must be transferred to institutions where concomitant cardiac procedures can be performed. In treating patients with WT, Doppler ultrasound must be used preoperatively in all cases, not only those in which clinical and radiologic signs of intravascular involvement are found. We propose that preoperative chemotherapy should be used, as it shrinks the thrombus and causes desirable adherence of the thrombus to the venous wall, reducing the probability of thromboembolism during the surgical procedure. We also find this method safer than in our 1st case, where neither cardiac arrest nor hypothermia was used. Our results agree with the literature that intracardiac extension of WT does not worsen its prognosis when a rational surgical approach is used. Accepted: 26 August 1999     

Gastroesophageal reflux—How to mend it?

Gastroesophageal reflux (GER) is a common condition affecting children. The clinical presentation varies widely from innocuous spitting up to life threatening apnea. Various diagnostic tests are available to document the etiology, presence or complications of GER. In most cases, conservative approach is sufficient. In complicated cases, pharmacotherapy is indicated while surgical therapy is reserved for resistant cases with complications.     

Respiratory distress in three newborns after mask disinfection with Endosporine. Probable role of glutaraldehyde]

BACKGROUND: Disinfectants for medical devices are uncommonly a cause of iatrogenic adverse effects. Nevertheless, when misused, they can induce severe complications. Three cases of acute respiratory distress in newborns probably induced by glutaraldehyde are reported. CASE REPORTS: Three children born by Caesarean section between 8 and 19 May 1999 in the same hospital presented acute respiratory distress requiring hospitalization in the neonatal intensive care unit; one child was premature. The clinical appearance, which was initially normal, deteriorated with a respiratory distress in 30 to 60 minutes. Recovery was uneventful in all cases. The diagnosis considered was a hyaline membrane disease. The enquiry conducted after this cluster onset identified, as a main contributing factor, the disinfection procedure recently introduced in the surgical theater. CONCLUSION: Review of toxicologic data on glutaraldehyde shows this is a highly irritating chemical for the respiratory tract, even at low concentrations. Clinical and radiologic features in these three neonates are compatible with a pulmonary sub-edema on an immature alveolar setting. The hypothesis proposed is that glutaraldehyde, the active ingredient of the biocidal formula used to disinfect the respiratory masks, was massively desorbed from the rubber and foam of which masks are made.     

Epiglottitis: incidence of extraepiglottic infection: report of 72 cases and review of the literature.

A review of 72 cases of epiglottitis seen at the Children's Hospital of Denver was undertaken to determine the incidence of extraepiglottic and septic foci in this disease. A parallel review of the literature was also undertaken. The clinical, bacteriologic, laboratory, and radiologic findings of this patient population are described. A 25% incidence of both pneumonia and cervical lymphadenitis was found to be associated with this illness. Exudative tonsillitis and otitis media were the only other complications, although they were infrequently noted. No cases of septic arthritis or meningitis were encountered, although 50% of these patients were recognized as bacteremic. A low incidence of septic complications (eight cases of meningitis and one case of periarticular abscess) is noted in a review of the literature. Recommendations for antibiotic management and definition of the population at risk for septic complications are given.     

Cysts of the omentum

Omental cyst is a rare lesion occurring primarily during childhood and young adulthood. It is closely related to mesenteric and retroperitoneal cysts, all conditions most likely being of congenital or benign neoplastic lymphatic origin. Omental cysts usually present as abdominal distention of long duration, less frequently as a palpable abdominal mass. Rarely they cause an acute abdominal surgical crisis clinically resembling appendicitis with peritonitis. Two cases are reported, one with acute and one with chronic symptoms. The clinical and radiologic features of omental cyst are reviewed in order to increase awareness of this entity.     

Uveitis in childhood

PURPOSE: To review the etiologic factors and complications of uveitis in patients younger than 16 years. PATIENTS AND METHODS: Between January 1989 and December 1999 in the Department of Ophthalmology of Hacettepe University School of Medicine, 219 patients were diagnosed or observed as having pediatric uveitis. After complete ocular and physical examinations, routine and specific laboratory and radiologic investigations were performed. Medical or surgical treatment was employed when necessary. RESULTS: Of the 219 patients, 112 were girls, with a mean age of 7.4 +/- 4.2 years, and 107 were boys, with a mean age of 8.3 +/- 3.4 years. In 24.2% of the cases, no etiologic factor could be ascertained; these cases comprised the idiopathic group. Among the remaining cases, the most common etiologies were toxoplasmosis, juvenile rheumatoid arthritis (JRA), pars planitis, Beh?et's disease, and Fuchs' heterochromic iridocyclitis. Anatomically, anterior uveitis was the most common form. The mean follow-up time was 37 +/- 6.2 months. Complications for which surgical treatment was employed were identified in 71 eyes (20.9%), most of which were due to JRA, pars planitis, or Beh?et's disease. CONCLUSION: Uveitis in childhood may be idiopathic or most commonly due to toxoplasmosis, JRA, and pars planitis. Due to inflammation itself or to prolonged therapy especially with corticosteroids, pediatric uveitis entities (mostly JRA, pars planitis, or Beh?et's disease) may result in complications necessitating a surgical approach.     

Ischemic stroke in children treated for acute lymphoblastic leukemia: a retrospective study

The clinical and diagnostic findings and the factors influencing the neurologic and radiologic outcome of symptomatic ischemic stroke were evaluated in a group of 2,318 children with acute lymphoblastic leukemia (ALL) treated according to the AIEOP (Italian Association of Pediatric Hematology and Oncology) study protocols. In this multicentric retrospective study, a questionnaire was sent to each of the 43 AIEOP centers participating in the study. The questionnaire was designed to obtain information on the number, type, and time of occurrence of ischemic strokes, biologic and immunologic features of each case, as well as clinical data of the recruited patients. A prevalence of 0.47% was found. All ischemic strokes were sinovenous thrombosis (SVT). The most common neurologic presentations were diffuse neurologic signs and seizures. MRI with or without venography revealed SVT in 100% of cases; superficial SVT was diagnosed in the majority of cases. Antithrombotic drugs, in particular unfractioned heparin and low-molecular-weight heparin, were administered without bleeding complications. This series shows an excellent long-term neurologic outcome in children with SVT. However, a complete radiologic resolution was found in only 54% of cases; the involvement of deep cerebral venous sinuses was associated with an unfavorable imaging outcome.     

Gastroesophageal reflux after the surgical treatment of esophageal atresia

The experience of the authors concerns 100 neonates treated for esophageal atresia between 1968 and 1980. A retrospective study of the sequelae observed in 73 survivors (age 3 to 15 years) found 26 cases of gastroesophageal reflux. This study demonstrates a high incidence of reflux in children who initially presented with a fistula and long gap esophageal atresia. It also shows that chronic respiratory disorders are more frequent in these patients and that esophagitis is the principal factor leading to stricture of the anastomosis stubborn to dilatation. In 14 patients, the severity of the clinical and endoscopic symptoms justified a surgical antireflux procedure, the only guarantee of clinical and radiologic recovery, with long term confirmation in 9 patients.     

先天性巨结肠再次手术的技术和技巧

先天性巨结肠是小儿外科常见消化道畸形之一,其手术技术已经相对成熟,但仍有少数患者因术后中远期并发症而接受再次手术.本文回顾近年发表的相关文献,并结合自身临床经验,对先天性巨结肠根治术后并发症的病因、如何避免并发症的发生以及再次手术技巧进行系统阐述.     

Multimodality imaging of an appendiceal intussusception

Appendiceal intussusception is a rare entity. The majority of cases reported in the literature address surgical and colonoscopic approaches to treatment of the condition. The existing radiologic literature largely describes the sonographic and double-contrast enema findings of appendiceal intussusception. We present a case of appendiceal intussusception and describe the air-contrast enema, sonographic and CT findings.     

Fetus in fetu: report of an additional, well-developed case

 An 8-month-old boy presenting with a fast-growing abdominal mass was operated upon to remove the tumor, which was confirmed to be a well-formed fetus in fetu. The authors describe the clinical, radiologic, and surgical findings and review the literature. Accepted: 11 May 2000     

MULTIPLE HEREDITARY GASTROINTESTINAL ATRESIAS: STUDY OF A FAMILY

ABSTRACT. Two siblings with multiple gastrointestinal atresias, from stomach to rectum, are reported. The pathological findings obtained from surgical material and complete autopsies are stressed. This syndrome, first described in 1973, presents a unique combination of clinical, radiologic and pathologic findings and is probably secondary to a malformative process taking place early in intrauterine life and effecting the whole gastrointestinal tract.     

A case report of hyperthyroid-associated thymic hypertrophy in a child,presenting as an anterior mediastinal mass

We present the case of an 8-year-old boy with an anterior mediastinal mass and signs of hyperthyroidism. The anterior mediastinal mass had radiologic characteristics suggestive of thymic hypertrophy and regressed with antithyroid therapy. Though thymic hypertrophy is a known manifestation of hyperthyroidism, this is the youngest reported case. In selected cases, the diagnosis may be made based on the clinical picture, radiologic appearance, and response to antithyroid therapy without the need of a thymic biopsy or thymectomy.     

Cerebrocostomandibular syndrome. Case report and literature review

Cerebrocostomandibular syndrome (CCM) is characterized by micrognathia, cleft palate, rib defects, and frequently, mental deficiency. Death from respiratory complications occurs in 40 percent of cases before they reach 1 year of age. We describe a case of CCM with the previously unreported findings of large for gestational age at birth, radiologic evidence of bilaterally displaced radial heads, and development of brachycephaly.     

Retrospective study of congenital hypertrophic pyloric stenosis and pylorospasm: Surgical vs. non-surgical treatment (author's transl)]

Congenital hypertrophic pyloric stenosis and pylorospasm have extensively been studied in 705 infants treated in 5 pediatric clinics in Hamburg, W.-Germany, during 1961 to 1970. With this report and its statistical evaluation we are aiming to provide basical information to answer the question still open to discussion: Are we allowed to demand surgical treatment in every single case of spastic hypertrophic pyloric stenosis? The following questions have been investigated: distribution of cases due to grade of severity; frequency of surgical and non-surgical, conservative treatment in these groups of different severity; its rate of success and of fatal outcome; length of clinical treatment and hospitalization; frequency of additional, intercurrent disease in both groups, with and without abdominal surgery; complications during and after surgery; late complications with regard to the way of treatment. From our results we conclude that surgical intervention is indicated in severe cases and in cases of medium severity, together amounting to 60% of all cases. In less severe, about 40%, cases non-surgical, conservative treatment is promising in almost all cases. The most important parameter to single out these less severe cases besides the general status is the roetgenologic finding that delayed emptying of the stomach starts within 15 to 20 min. Important as well is a balanced electrolyte and acid-base metabolism. Once more we advocate individual and subtle decision of paramount surgical intervention, reasoning from the background of this study where the percentage of infants successfully treated by non-surgical, conservative medical ways is very high.     

Osteochondroma with metaphyseal abnormalities after total body irradiation followed by stem cell transplantation

The occurrence of osteochondroma after total body irradiation (TBI) followed by stem cell transplantation (SCT) in our institutions was described, and its clinical significance discussed. Of 305 cases treated with SCT using TBI conditioning from 1980 to 2001, 4 cases of osteochondroma were identified on clinical examination. Mean age at the time of TBI was 4.4 years (range, 1.6 to 8.0). One patient developed multiple osteochondromas. All 4 cases showed metaphyseal abnormalities, including sclerotic metaphyseal lesion, fraying, and longitudinal striation, in the area where osteochondromas occurred. Only 1 patient required resection of the tumor due to pain. Two cases had other skeletal abnormalities including slipped capital femoral epiphysis and valgus-knee deformity, which required surgical intervention to prevent or correct these deformities. Osteochondroma is one of the complications developing after TBI, possibly concurrently with the metaphyseal abnormalities as seen on radiographs. However, clinical problems arising from osteochondroma are minimal, and surgical intervention is necessary in limited cases.     

儿童脊髓纵裂手术治疗的并发症

目的：研究脊髓纵裂手术并发症，提高手术疗效，提出预防措施。方法：我院自1978年8月至2001年7月手术治疗脊髓纵裂患儿39例，6例出现不同程度并发症，发生脑脊液漏2例、神经损伤3例、硬膜外血肿1例。对6例并发症的原因、时间、治疗、预后、临床表现进行分析。结果：6例并发症产生原因是由于术中硬膜的修补不完善、损伤及牵拉脊髓，止血不严密造成，经治疗预后较好。结论：手术中熟悉脊髓纵裂解剖特点，显微操作，严密止血可避免并发症。     

Renal upper pole calyceal obstruction: clinical and radiological significance (author's transl)

Obstruction of the superior renal calyces due to intrarenal vascular compressions is assumed on the basis of a characteristic sharply defined oblique filling defect with or without blunting of the fornices. Scintillation camera renography shows prolonged retention in the superior collecting system of the involved kidney. 11 out of 23 children had a symptomatology of recurrent hematuria, other origins of hematuria were excluded. 5 patients had urinary tract infection, the remaining 7 had normal urinary findings. Renal function was always normal. There is no correlation between severity of blunting and hematuria. In contrast to other studies which included only patients with blunting and ectasis even cases without blunting of the fornices have a clinical symptomatology. Deterioration of radiologic appearance and kidney function was not found. Any idiopathic hematuria should be investigated for calyceal obstruction. Uncomplicated cases require no therapy, long term follow-ups with regard to complications such as urinary tract infection and urolithiasis are indicated.     

Necrotizing enterocolitis in children between the ages of 48 days and 24 months

Necrotizing enterocolitis (NEC) is the pre-eminent gastrointestinal disorder encountered among stressed premature infants in neonatal intensive care units. Thirty-one sporadic cases of gastroenteritis occured in children between the ages of 48 days and 24 months who presented with varying degrees of septicemia, hypovolemic shock, hypotension, metabolic acidosis, and disseminated intravascular coagulation. These patients developed clinical features and radiologic signs characteristic of neonatal NEC. The occurence of NEC in children as a sequel to gastroenteritis and its etiology, pathogenesis, clinical features, laboratory data, radiologic findings, and outcome are discussed.