Wall In Children Research Articles

Component Separation to Salvage a Renal Allograft

Primary closure of the abdominal wall in children following renal transplantation may be challenging, especially if the transplanted kidney restricts the abdominal domain. Pressure on the graft can be further exacerbated by bowel edema leading to abdominal compartment syndrome (ACS) and graft compromise (1). The subject of this report is an 11-year-old female patient whose past medical history was significant for a previous living-related renal transplant lost because of noncompliance and acute rejection, and several laparotomies for intestinal intussusception and adhesive intestinal obstruction. A kidney from a 16-year-old donor was accepted for her second transplant. The transplant was performed through a midline laparotomy. At exploration, a “frozen abdomen” was found. The first kidney graft was found in the right lower abdomen. A decision was made to sew the new graft to the left iliac vessels and urinary bladder. The transplant was straightforward and followed by a primary abdominal closure. Brisk diuresis continued for the first six hours but gradually decreased to a plateau of 10 mL/min. A Doppler ultrasound exam of the kidney found a decreased flow in the iliac vein while the arterial flow to the graft was normal. An urgent contrast venogram showed external compression of the iliac vein. An emergency exploration found that the kidney was edematous and cyanotic, but it rapidly improved as the abdominal domain was increased. A decision was made to avoid a prosthetic abdominal closure and perform a component separation instead. The external oblique muscles were exposed and transected bilaterally from the costal arch to the iliac crests (2), allowing a tension-free fascial closure. On-table venogram showed no further compression of the iliac vein. The urine output increased to 100 mL/hour within 24 hours; the serum creatinine reached a reference range in 48 hours. The patient was free of complaints six months after the procedure and had an intact abdominal closure and normal kidney function. Prolonged increase of the intra-abdominal venous pressure reduces the perfusion pressure and results in ACS, a vicious circle of reduced venous outflow, tissue ischemia, and edema (3), which impairs all systems including the renal system (4). ACS has infrequently been reported in renal transplant. Four previous studies describing seven patients (1996–2003) were quoted by a recently published report of 11 ACS patients (4). ACS typically presented with renal disfunction (4, 5), similar to our observation. Our recognition of ACS was based on clinical criteria such as urine output and observation of the graft perfusion. Ultrasound Doppler of the interlobar and segmental renal arteries showing absent or reversed diastolic blood flow (4, 5) was not observed in our patient. Treatment of ACS consists of intraperitonealization (3, 4) with or without fasciotomy (4), or prosthetic bridging (6–8) if the kidney is extraperitoneal. There is one previous report of a pediatric intra-abdominal renal transplant treated with decompressive fasciotomy (5) and another report describing a prosthetic fascial bridge, which prevented ACS (9). Our report is the third in the pediatric setting. We propose component separation as a definitive solution to obtain a tension-free abdominal closure. Julian E. Losanoff Department of Surgery Wayne State University and John D. Dingell VAMC Detroit, MI Trevor W. Reichman Marc R. Garfinkel Giuliano Testa J. Michael Millis Department of Surgery University of Chicago Chicago, IL

A new method for estimating voice sounds transmitted to the chest wall in children and adolescents

A new method for estimating voice sound transmission to the chest wall is proposed. The spectral characteristics of voice-transmitted sounds have been estimated in children and adolescents. A total of 37 subjects aged 7–17 yers were examined. The frequency of the first spectral peak of the voice-transmitted sounds “tree, tree” (M ± SD) is 263.7 ± 7.82 and 253 ± 4.29 Hz at ages of 7–11 and 12–14 years, respectively. In male adolescents aged 15–17 years, this frequency is decreased to 100–150 Hz. The slope of the descending segment of the spectrum on the high-frequency side of the peak steadily increases on moving from the upper to the lower zones of the lungs, which may be related to the increase in the air content of the lung tissue. The difference between the amplitudes of the first and second spectral peaks of voice-transmitted sounds over symmetric regions of the chest on the right and left sides (Me(Q75-Q25)) is −0.1(11.0) dB and does not depend on age or sex, which can be interpreted as an average statistical symmetry of sound transmission.

Optimal site for throat culture: tonsillar surface versus posterior pharyngeal wall

To determine the optimal site of throat culture for the detection of potential pathogens by comparing culture results from the tonsillar surface and the posterior pharyngeal wall in children selected for adenotonsillectomy and in children without upper respiratory disease. Cotton culture swabs were taken from the tonsillar surface and the posterior pharyngeal wall of 50 children selected for adenotonsillectomy for symptoms of recurrent tonsillitis and/or adenotonsillar hypertrophy and of 50 children without upper respiratory disease. Potential respiratory pathogens were identified. In the overall group (n = 100), positive culture results were found in 67 posterior pharyngeal wall samples and 47 tonsillar surface samples (P = 0.001). Haemophilus influenzae was the most frequently isolated micro-organism both in the posterior pharyngeal wall and the tonsillar surface samples; 55 and 35%, respectively (P = 0.001). Group A beta-haemolytic streptococci were found in the samples of the posterior pharyngeal wall and the tonsillar surface in 17 and 13%, respectively (P = 0.2). When dealing with patients with sore throat, sampling both tonsillar surfaces is enough for the detection of group A beta-haemolytic streptococci. When detection of other bacteria is also important, such as for research purposes, the posterior pharyngeal wall should be sampled as well.

A Simple and Secure Method to Fix Laparoscopic Trocars in Children

We introduce a simple method of fixing trocars to the abdominal wall in children. Before anchoring the trocar, a piece of Tegaderm polyurethrane adhesive (3M Healthcare, St. Paul, Minnesota) is attached to the trocar. A silk stitch is anchored to neighboring skin, and then transfixed over the shaft of the trocar through the adhesive. Both inward and outward movement of the trocar can be restrained. This method is simple, fast, secure, and can be applied to trocars of any size.

Strangulated umbilical hernias in children

Umbilical hernia is a frequent pathology of the anterior abdominal wall in children. The hernia ring closes usually before 4 years, but a strangulation can occur. It is an exceptional complication according to the literature data. Since this complication is rare, we undertook a retrospective study of these strangulations in a 5-year period from January 1997 to December 2001 at Aristide Le Dantec hospital. We collected 41 cases that underwent emergency surgery operations for strangulated umbilical hernias, which represent about 15% of umbilical hernias operated during the same period. The age range was 8 months to 10 years and the average age was 14 months. All the children were examined within 24 h after the onset of the disease and the surgery was immediately performed. In five cases the bowel was necrosed and perforated and we performed a resection followed immediately by an anastomosis. In the follow up, two children presented wound infections and a hernia recurred in one child. There was no mortality. This study conducts us to question western reports which recommend conservative therapy for umbilical hernia in children.

Comparison of the Ratio of Upper to Lower Chest Wall in Children with Spastic Quadriplegic Cerebral Palsy and Normally Developed Children

The upper chest wall does not grow properly in children with spinal muscular atrophy (SMA) with paradoxical breathing. This suggests that long-term inability to take a deep breath in developing children may result in underdevelopment of the upper chest wall. In addition, a rapid and paradoxical breathing pattern is frequently observed in children with severe cerebral palsy (CP), which often corresponds to the underdevelopment of the upper chest wall. The present study is designed to evaluate the ratio of the upper to lower chest wall in children with severe spastic quadriplegic CP, compared with normal children. We compared normal children with children that had spastic quadriplegic CP who did not have kyphosis or scoliosis. Test subjects were matched in terms of age, height, and weight. The diameters of upper chest (Dapex) and of lower chest (Dbase) were measured on the anteroposterior (AP) view of a chest X-ray and the Dapex to Dbase ratio was calculated. In selected cases the forced vital capacity (FVC) was measured using a Wright Respirometer. The Dapex to Dbase ratio was significantly lower in the CP group than in the control group (p<0.001). The ratio increased linearly with age (p<0.001) in both CP (R = 0.372) and control groups (R = 0.477). The FVC/preFVC showed significant correlation with the Dapex to Dbase ratio (R = 0.542, p<0.01). The results of this study suggest a deviation of optimal chest wall structure in children with spastic quadriplegic CP.

The diagnosis and management of children with blunt injury of the chest

Thoracic trauma remains a major source of morbidity and mortality in injured children, and is second only to brain injuries as a cause of death. The presence of a chest injury increases an injured child’s mortality by 20-fold. Greater than 80% of chest injuries in children are secondary to blunt trauma. The compliant chest wall in children makes pulmonary contusions and rib fractures the most common chest injuries in children. Injuries to the great vessels, esophagus, and diaphragm are rare. Failure to promptly diagnose and treat these injuries results in increased morbidity and mortality.

The distribution of dividing T cells throughout the intestinal wall in inflammatory bowel disease (IBD).

T cell hypersensitivity has been implicated in the tissue damage in Crohn's disease (CD). All studies to date have examined mucosal T cells, although much of the tissue damage occurs in the submucosa and muscle layers. The aim of this work was to study T cell proliferation throughout the intestinal wall in children with IBD. Surgical resection material from 19 children with CD (10 ileal, 10 colonic), seven with ulcerative colitis (UC), and 12 normal controls was studied. The distribution of dividing T cells was investigated by double-immunohistochemistry using Ki67 to identify proliferating cells, and CD3 to identify T cells. In ileal and colonic lamina propria virtually no Ki67+, CD3+ cells were seen in control, UC or CD tissue. In contrast, there were significantly more Ki67+, CD3+ cells within the lymphoid follicles of ileal and colonic CD than in the follicles in UC and controls. Increased numbers of Ki67+, CD3+ cells were present in the submucosa, muscle layers (M) and serosa in Crohn's ileitis and colitis compared with the lamina propria (LP), although only in the muscle of the colon was the difference statistically significant (LP, 0.4% (0-1%); M, 1.6% (0-5.2%); P = 0.03). Pooling data from ileal and colonic CD, however, did show significantly increased Ki67+, CD3+ cells in both serosa and muscle layers compared with the LP. Dividing T cells have been identified in the deeper layers of the gut wall in CD. These may contribute to the fibrosis and muscle hyperplasia characteristic of the condition.

Pectoralis muscle flap reconstruction of pediatric sternal wound infections.

Sternal wound infection (SWI) is a life-threatening complication in both the pediatric and adult population. The morbidity and mortality of SWIs have decreased with the use of muscle flap reconstruction of the chest wall. Although the pectoralis muscle flap is the most frequently used flap in adults for reconstruction after SWI, its use in children has not been well described. To review current experience with the use of the pectoralis muscle flap in a pediatric population, a retrospective review of 1,200 consecutive median sternotomies occurring at the Children's Hospital of Pittsburgh between 1992 and 1997 was performed. All cases of postoperative SWI were identified, and the operative management was performed by a single surgeon. Reconstruction was performed with the use of unilateral or bilateral pectoral muscle flap advancements. Sternal wound infections developed in nine patients (0.75% incidence). The mean duration from initial sternotomy to the time of presentation of deep SWI was 39.5 days. Eight patients underwent reconstruction using pectoralis muscle advancement flaps. Two patients (25%) were reconstructed with a unilateral pectoralis advancement flap, whereas six patients (75%) required bilateral advancement flaps. One patient was treated with conservative management. The average length of stay after reconstruction was 9 +/- 4 days. Duration of follow-up ranged from 3 to 42 months (mean: 16 +/- 12 months). All reconstructions ultimately resulted in well-healed wounds with satisfactory cosmesis. No developmental or functional deficits have been documented in follow-up visits. Sternal wound infection is a serious postoperative complication of median sternotomy. Aggressive operative management with the use of muscle flap reconstruction has helped to lower the morbidity and mortality of this infection. The successful use of pectoralis muscle flap advancement for functional and esthetic reconstruction of the chest wall in children is described.

Ewing's Sarcoma/Primitive Neuroectodermal Tumor of the Chest Wall

Ewing's sarcoma/primitive neuroectodermal tumor is the most common tumor of the chest wall in children and adolescents. It is extremely malignant with a high frequency of both metastatic spread and of local recurrence. Cure requires intensive therapy to control both distant and local disease. Surgery and high-dose radiotherapy can achieve equivalent local control; however, radiation is associated with the additional morbidities of second malignancy and a significant adverse impact on both cardiac and pulmonary function. The optimal therapeutic sequence is initial biopsy followed by induction chemotherapy with subsequent resection of the primary tumor. This approach will achieve the lowest incidence of tumor present at the margins of resection and, hence, need for postoperative radiotherapy. The chest wall is a rare site for tumors in children and adolescents. In a series reported from St Jude's Children's Research Hospital, chest wall tumors constituted only 1.8% of the solid childhood tumors. They are primarily mesenchymal in origin and the Ewing's sarcoma/primitive neuroectodermal tumors (PNET) predominate. This report concentrates on the later tumors. They are recognized to be extremely malignant, and cure in those who present with metastatic disease is very difficult to achieve. Recent advances in our understanding of their cytogenetic basis and optimal treatment are presented.

Funnel chest: treatment strategy and follow-up

Although funnel chest is the most frequently seen deformity of the anterior chest wall in children, there is still considerable controversy regarding three major aspects, namely, the frequency of such deformities, their physiological importance, and the methods available for treatment. We retrospectively analyzed our experience with the 154 patients managed in our department. In 81 of these an operation was performed (OP), and the clinical findings for this group were compared with the 73 patients in whom an operation was not performed (NOP). Evaluation included subjective findings, especially the views of the patients' parents, and objective findings, including chest radiographs, computed tomography (CT), spirogram, electrocardiography, and echocardiography. In all patients the assessment included postoperative respiratory symptoms, appearance of the chest, and psychological aspects related to the deformity. Post-operatively, respiratory symptoms almost invariably subsided. The cosmetic result could initially be regarded as satisfactory or fair during the first 10 years following surgery, but over time there was frequently increasing concern regarding the scar. The NOP patients showed significantly less severity of the funnel index compared with OP patients. However, there was no spontaneous improvement in the deformity in older patients; most of the NOP patients continued to show a cosmetic deformity and 26.7% had psychological problems. This retrospective study confirms that our treatment strategy of objective criteria for operation (functional compression index > 0.2, % vital capacity <80, and CT index less than 0.25) and timing of operation (between 4 and 6 years of age) provides good results. Based on the analysis of long-term follow-up, surgery is considered indicated in patients with severe deformity. However, in the interest of psychological development, the indications for surgery may be extended.

Repair of pectus excavatum deformities in children: a new perspective of treatment using minimal access surgical technique.

Minimally invasive correction of pectus excavatum (PE) deformities of the anterior chest wall in children is safe and effective. Prospective cohort study. Tertiary pediatric referral center. Between February 1996 and July 2000, 36 patients underwent minimally invasive repair (MIR) of PE deformities, and 6 patients had traditional Ravitch repairs (RR). Morbidity, operating time, estimated blood loss, days to tolerating a regular diet, and length of hospital stay. Thirty-six children underwent MIR for moderate to severe chest wall deformities, with a mean operative time of 1.6 hours, a mean blood loss of 22 mL, a mean time to tolerating a regular diet of 2.9 days, no intensive care unit admissions, and a mean length of hospital stay of 5.5 days. Six children had RR procedures performed for moderate to severe deformity, with a mean operative time of 5.2 hours, a mean blood loss of 222 mL, a mean time to tolerating a regular diet of 3.3 days, 2 patients admitted to the intensive care unit, and a mean length of hospital stay of 4.5 days. Complications for both procedures consisted mainly of postoperative pneumothorax. The MIR technique for PE is less invasive, less morbid, and better tolerated than traditional open RR of this common pediatric chest wall deformity. Elective surgical reconstruction can be safely performed in children rather than limiting repair to only symptomatic patients with severe deformities.

Bronchial Rupture after Direct Chest Trauma in a Child

Tracheobronchial injuries are rare in trauma patients, and most often occur after motor vehicle accidents. Occasionally, other mechanisms cause airway disruption. The pliability of the chest wall in children greatly adds to the differences in injuries when compared with adult trauma patients. We present the case of a six-year old girl with an isolated right-sided bronchial rupture after direct trauma to the chest.

Thickening of the renal collecting system wall in children

Thickening of the renal collecting system wall in children

Arterial mechanical changes in children with familial hypercholesterolemia.

Atherosclerosis is preceded by a phase of changes in the arterial wall that could have functional consequences even before the appearance of atheromatous changes. We hypothesized that early alterations of the mechanical properties of the arterial wall could precede clinical and echographic modifications. We used an automatic, computerized, ultrasonic procedure to evaluate geometric and mechanical characteristics of the common carotid artery (CCA) in normotensive children with primary familial class IIA hypercholesterolemia (FH; n=30; mean+/-SD age, 11+/-2 years old; mean+/-SD systolic/diastolic blood pressure, 109+/-9/55+/-7 mm Hg). These subjects were compared with age-matched, nonobese control subjects (n=27; 11+/-3 years old; 112+/-10/55+/-7 mm Hg). Noninvasive ultrasonic measurements were performed by the same investigator to measure the CCA luminal systolic and diastolic diameters and intima-media thickness (IMT). The cross-sectional compliance, cross-sectional distensibility, and the incremental elastic modulus of the CCA wall were then calculated. Finally, we assessed the degree of reactive hyperemia in the brachial artery produced after distal cuff occlusion and release. The changes in brachial arterial diameter in response to reactive hyperemia (endothelium-dependent dilation) and to glyceryltrinitrate (endothelium-independent dilation) were then measured. In patients with FH, we observed a significant reduction of systodiastolic variations in diameter (by 20%, P:<0.001) without a significant difference in IMT. Cross-sectional compliance and cross-sectional distensibility were significantly reduced in FH subjects (by 15%, P:<0.05 and 19%, P:<0.01, respectively). In parallel, the incremental elastic modulus was significantly increased (by 27%, P:<0.01) in children with FH. No correlation was evident between the carotid incremental modulus and either IMT or plasma low density lipoprotein cholesterol level. There was no difference in diameter of the brachial artery at rest in control and FH subjects (3.0+/-0.5 versus 3.0+/-0.4 mm). The reactive hyperemia and glyceryltrinitrate dilation were also similar in the 2 groups. However, the flow-mediated dilation of the brachial artery was smaller in the FH subjects (4.2+/-2.9%) than in controls (9.0+/-3.1%, P:<0.001). In FH, endothelium-dependent dilation was negatively correlated with the plasma low density lipoprotein cholesterol level (P:<0.04). These results indicate that increased stiffness of the CCA wall in children with FH is independent of blood pressure and could be related to endothelial dysfunction. Thus, alterations in CCA wall mechanics could be early and easily measurable markers of atheromatous changes in the arterial wall.

3710 HIGH RESOLUTION PROBE ENDOSONOGRAPHY IN ALLERGIC ESOPHAGITIS.

Allergic esophagitis (AE) is an inflammatory disorder of the esophagus characterized by lack of response to medical and surgical anti-reflux therapies, normal esophageal pH recording, distinctive mucosal histopathology, and response to anti-allergic treatments (Am J Surg Pathol, 1999;23:390-6). Dysphagia and food impaction occur frequently, despite the absence of stricture, and resolve with medical therapy. The pathophysiology of this dysphagia is unknown but may relate to reversible changes in the esophageal wall. High-resolution probe endosonography (HRPE) provides detailed radial imaging and permits real-time measurement of individual layers of the esophageal wall.We have applied HRPE to investigate changes in children with AE and report our initial findings. Methods: Children with AE and control children without esophagitis were enrolled for study. The Olympus EU-M30 ultrasound processor and 20 MHz miniprobe were utilized. Probes were passed through the operating channel of a gastroscope at the time of diagnostic endoscopy. Small amounts of water were infused into the esophageal lumen for acoustic coupling. Duplicate real-time measurements (0.1mm resolution) of the esophagus were obtained with the probe tip positioned 3-5 cm above the lower esophageal sphincter and with the esophagus in a non-contracted state. Measurements were taken from areas of maximal thickness where all of the echo layers could be clearly distinguished and included thickness of the total wall (tw), combined mucosa and submucosa (m/sm), and muscularis propria (mp). Comparisons were made with control patients without esophagitis. The Mann-Whitney test was used for non-parametric statistical analysis of mean values. Results: Eight children with AE (mean age, 10.1 ± 5.2yr) were compared with four control children (mean age, 7.8 ± 6.3yr)(p=0.396). Seven echo layers were seen in all patients. Echo layers were significantly thicker in AE patients compared with control patients. Mean tw thickness was 2.2 ± 0.1mm (control) compared with 2.8 ± 0.7mm (AE)(p=0.035). Mean m/sm thickness was 1.1 ± 0.2mm (control) versus 1.6 ± 0.4mm (AE)(p=0.017). Mean mp thickness was 1.0 ± 0.1mm (control) compared with 1.3 ± 0.3mm (AE)(p=0.025). Conclusion: HRPE identifies significant thickening of the mucosa/submucosal and muscle layers of esophageal wall in children with allergic esophagitis compared to normal controls. This thickening may contribute to the observed dysmotility in these patients. Allergic esophagitis (AE) is an inflammatory disorder of the esophagus characterized by lack of response to medical and surgical anti-reflux therapies, normal esophageal pH recording, distinctive mucosal histopathology, and response to anti-allergic treatments (Am J Surg Pathol, 1999;23:390-6). Dysphagia and food impaction occur frequently, despite the absence of stricture, and resolve with medical therapy. The pathophysiology of this dysphagia is unknown but may relate to reversible changes in the esophageal wall. High-resolution probe endosonography (HRPE) provides detailed radial imaging and permits real-time measurement of individual layers of the esophageal wall.We have applied HRPE to investigate changes in children with AE and report our initial findings. Methods: Children with AE and control children without esophagitis were enrolled for study. The Olympus EU-M30 ultrasound processor and 20 MHz miniprobe were utilized. Probes were passed through the operating channel of a gastroscope at the time of diagnostic endoscopy. Small amounts of water were infused into the esophageal lumen for acoustic coupling. Duplicate real-time measurements (0.1mm resolution) of the esophagus were obtained with the probe tip positioned 3-5 cm above the lower esophageal sphincter and with the esophagus in a non-contracted state. Measurements were taken from areas of maximal thickness where all of the echo layers could be clearly distinguished and included thickness of the total wall (tw), combined mucosa and submucosa (m/sm), and muscularis propria (mp). Comparisons were made with control patients without esophagitis. The Mann-Whitney test was used for non-parametric statistical analysis of mean values. Results: Eight children with AE (mean age, 10.1 ± 5.2yr) were compared with four control children (mean age, 7.8 ± 6.3yr)(p=0.396). Seven echo layers were seen in all patients. Echo layers were significantly thicker in AE patients compared with control patients. Mean tw thickness was 2.2 ± 0.1mm (control) compared with 2.8 ± 0.7mm (AE)(p=0.035). Mean m/sm thickness was 1.1 ± 0.2mm (control) versus 1.6 ± 0.4mm (AE)(p=0.017). Mean mp thickness was 1.0 ± 0.1mm (control) compared with 1.3 ± 0.3mm (AE)(p=0.025). Conclusion: HRPE identifies significant thickening of the mucosa/submucosal and muscle layers of esophageal wall in children with allergic esophagitis compared to normal controls. This thickening may contribute to the observed dysmotility in these patients.

High Frequency Compression of the Thoracic Wall in Children With Bronchiolitis

High Frequency Compression of the Thoracic Wall in Children With Bronchiolitis

#47 Primitive neuroectodermal tumor of the chest wall in children and young adults

#47 Primitive neuroectodermal tumor of the chest wall in children and young adults

Laparoscopic cholecystectomy with abdominal wall traction in pediatric patients

Laparoscopic cholecystectomy using abdominal wall traction with a winch retractor instead of a pneumoperitoneum in pediatric patients is reported. Lifting the abdominal wall by the falciform ligament is useful for laparoscopic procedures, especially in small children. Because of the elasticity of the abdominal wall in children, traction on the right upper quadrant alone allows sufficient access to perform a cholecystectomy without maintenance of a pneumoperitoneum.

Malignant small round cell tumor (Ewing's-PNET) of the chest wall in children

From 1976 to 1989 the authors treated 21 infants and children with malignant tumors of the chest wall. Fifteen were classified as Askin's tumors or Ewing's sarcoma, which we now consider as a single entity of primitive neuroectodermal origin. They are infrequent but highly aggressive tumors that involve the chest wall in children. Five patients presented with metastatic disease; despite chemotherapy and radiotherapy, all succumbed to progressive disease. Ten patients with localized disease received combined modality therapy including surgical resection (three after initial chemotherapy), radiotherapy, and chemotherapy. Surgery involved resection of the mass and up to three ribs, with prosthetic mesh reconstruction in one patient. Six of the 10 patients with localized disease are continuously disease-free 3.5 to 9 years (median, 5 years) following diagnosis. No patient had local recurrence. Of the four whose treatment failed, one died (free of disease) from complications after resection of an extensive primary tumor. In the second patient, acute monocytic leukemia developed shortly after relapse in a distant bone site. The patient died during induction for the leukemia. In the other two patients, hilar and carinal lymph node relapse occurred 68 and 80 months after initial treatment. One of the patients is considered in second remission (now 105 months later) after further chemotherapy (Adria-VAC) and radiation; the other succumbed to secondary relapse 17 months after second remission was achieved through chemotherapy, radiation, and surgery. Initial percutaneous needle biopsy of the large lesions has provided adequate material for histological, immunohistochemical, cytogenetic, and ultrastructural diagnosis, and permitted initial chemotherapy before proceeding to surgical resection. Preliminary chemotherapy was very advantageous in shrinking the tumor and markedly decreasing its vascularity. In fact, no viable tumor was identified in the specimens resected after initial chemotherapy in three of five children (two with metastases). Resection of the primary tumor before radiotherapy allows accurate marking of the area of involvement to permit limited radiation of the thorax. With this intensive multimodality therapy, the authors have achieved a predicted life-table survival of 53% in patients without metastatic disease at presentation, and there has been no local chest wall recurrence.