Mediastinal tumors in children: a single institution experience.

Mediastinal masses in children are a heterogeneous group of asymptomatic or potentially life-threatening congenital, infectious, or neoplastic lesions that present complex diagnostic and therapeutic dilemmas. Some patients are asymptomatic; in others, the mass may compress mediastinal structures and cause sudden asphyxia. In these cases, close cooperation is needed among pediatric surgeons, anesthesiologists, intensivists, oncologists, and radiologists. The files of 45 children with mediastinal masses admitted between 1986 and 1999 to the Pediatric Intensive Care Unit (PICU) of Schneider Children's Medical Center of Israel were reviewed. Twenty-one were admitted for perioperative care, and 21 for emergency care, including 19 with respiratory distress. Five of the emergency care group had asphyxia and 10 needed assisted ventilation. Two children were admitted for evaluation and 1 for leukopheresis. The children admitted on an emergency basis had more clinical findings than the postoperative group: almost 80% had dyspnea and more than 45% had oxygen desaturation; 33% had cough and noisy breathing, and 25%, superior vena cava syndrome or hepatosplenomegaly. Eight patients (17.8%) had benign disease and 37 (82.2%) malignant disease. The patients with a benign mass were significantly younger than the patients with a malignant mass (p<0.005); in 5 cases (12.5%), a congenital anomaly presented as a mediastinal mass. Most of the malignant masses were of hematologic origin (40.5%), followed by neurogenic tumors (27%). Twenty-seven patients underwent surgery, including 6 emergency procedures (3 partial resections, 2 biopsies, 1 lymph node biopsy). There were no intraoperative or postoperative deaths. The present series emphasizes the complex care children with a mediastinal mass require. They should be treated in a tertiary center with a multidisciplinary approach.

Skin level division of percutaneous endoscopic gastrostomy without endoscopy retrieval: a hazardous procedure.

Percutaneous endoscopic gastrostomy (PEG) has become the method of choice for long-term enteral access in the pediatric population. Since its introduction, several common complications have been described. Less well known is the danger of removing or replacing a PEG by cutting the device at skin level without endoscopic assistance to ensure the complete removal of all parts. The aim of the present work is to describe a patient in whom gastrostomy parts were retained after PEG removal, causing bowel obstruction and perforation.

A rare case of colonic obstruction by 'cherry tomato' phytobezoar: A simple technique to avoid enterotomy.

Small bowel obstruction is the most common complication of phytobezoar in children. The authors present a rare case of colonic obstruction caused by a cherry tomato phytobezoar in a 16-month-old child that was treated successfully during laparotomy after failure of external fragmentation.

Infantile acute pancreatitis after mumps vaccination simulating an acute abdomen.

We describe an extremely rare case of acute pancreatitis presenting as an acute abdomen that appeared as a complication of mumps vaccination in a young child. A laparotomy performed because of suspected perforated appendicitis proved unnecessary in retrospect. No similar case in infancy and early childhood has been reported to date.

A simple method of intraoperative confirmation of intestinal patency.

Routine intraoperative rectal temperature monitoring may serve in addition as a means of distal intestinal patency confirmation. A simple method, which is of immense importance especially when operating on infants and small children after NEC or intestinal atresia, is described.

Small bowel obstruction and covered perforation in childhood caused by bizarre bezoars and foreign bodies.

BACKGROUND: Small bowel obstruction with perforation is an unusual and rare complication of bezoars. OBJECTIVE: To describe our use of emergency laparotomy to treat intestinal obstruction caused by bizarre bezoars. CONCLUSIONS: An aggressive surgical approach to intestinal obstruction in the pediatric disabled or mentally retarded population is recommended.

High frequency of loss of heterozygosity for 1p35-p36 (D1S247) in Wilms tumor.

We analyzed the loss of heterozygosity (LOH) for 1p in 18 Wilms tumors using a panel of 11 polymorphic markers. Loss of heterozygosity was identified in 56% of the tumors. The smallest region of overlap was defined for marker D1S247, underlying the 1p35-1p36.1 locus. This is the highest LOH frequency for 1p, or for the well-defined 11p13 and 11p15.5 loci. Based on the fact that tumors of all stages, with both favorable and unfavorable histology, exhibited LOH, we suggest that the 1p35-1p36.1 locus is involved in the etiology of Wilms tumor.

Minimally invasive surgery in pediatric endocrinology.

The use of minimally invasive surgery (MIS) in children and adolescents is steadily increasing. The aim of the present review was to summarize the status of MIS in pediatric endocrinology. We found that laparoscopic procedures have been proven useful for the diagnosis or treatment of endometriosis and its associated manifestations, undescended testicles, ambiguous genitalia, adnexal torsion and ovarian cyst. Considering the safety and efficacy of these applications, the more rapid recovery of the patients, and the considerably less pain induced, we believe MIS will gradually take precedence over standard procedures in many areas of endocrinology in the young population.

Ovarian masses in children.

We evaluated the outcome of children with ovarian mass operated on at our Center over an 8-year period. Thirty-four girls aged 1 day to 17 years were included in the study. Mean duration of follow-up was 39.5 months. Eighteen had a nonneoplastic mass and 16 a neoplastic mass, eight of which were malignant. Patients with a malignant tumor underwent adnexectomy of the affected side and appendectomy, without removal of the uterus or the other ovary and without partial omentectomy; only the one girl with bilateral malignant disease had bilateral adnexectomy. Five of the eight patients with malignant disease received chemotherapy. All patients are alive with no evidence of disease. Pediatric ovarian masses are rare but have a relatively high rate of malignancy. They differ from adult malignant tumors in many aspects. Conservative surgery should be applied to preserve fertility and combined, if necessary, with aggressive chemotherapy. A good prognosis may be expected in most cases, even with progressive disease.

A plea for incidental appendectomy in pediatric patients with malignancy.

The evaluation of right lower quadrant (RLQ) abdominal pain in pediatric patients with malignancy can be difficult. However, since the mortality rate from peritoneal infections in these patients is very high, the differential diagnosis of RLQ peritoneal irritation, mainly of acute appendicitis (AA) versus neutropenic enterocolitis (NE), is crucial. Three cases of pediatric patients with malignancy demonstrating these difficulties are represented to enlighten this problem. The first patient died of multiorgan failure after operation for perforated appendicitis without generalized peritonitis. The second had a severe life-threatening postoperative complication because of delayed diagnosis of acute appendicitis. The third patient with malignant pelvic spread, underwent an unnecessary abdominal exploration for suspected AA. In all these cases and probably in many others, the clinical outcome could have been different if a previous incidental appendectomy had been performed during the primary abdominal operation. Incidental appendectomy in oncologic patients is recommended to facilitate the differential diagnosis of RLQ pain and to exclude the diagnosis of AA.

Perineal reconstruction for severe sequela of ecthyma gangrenosum: report of a case.

Ecthyma gangrenosum is a cutaneous gangrenous disorder which usually follows Pseudomona aeruginosa infection and is found mainly in immunosuppressed children. We describe a case of a five-year-old female with leukemia with a severe perineal ecthyma gangrenosum resulting in a cloaca-like deformity. One year later a perineoplasty with puborectalis interposition and overlapping external anal sphincteroplasty was successfully performed, achieving satisfactory continence.

Spontaneous splenic rupture in infectious mononucleosis: conservative management with gradual percutaneous drainage of a subcapsular hematoma.

Spontaneous splenic rupture (SSR) is a rare but potentially lethal complication of infectious mononucleosis (IM). Because the inflamed spleen is usually enlarged, congested, and friable, emergency splenectomy is recommended. We describe the conservative management of a SSR in a 16-year-old boy with IM. A pigtail catheter was inserted under ultrasonographic guidance and left in place for 36 h. This allowed the successful evacuation of the hematoma without compromising the splenic parenchyma.

Splenic "regeneration" after partial splenectomy for Gaucher disease: histological features.

Partial splenectomy for Gaucher disease is often followed by reenlargement of the splenic remnant. It remains unclear if this process is due to tissue regeneration or to continued deposition of glucocerebroside in the reticuloendothelial system or both. We compared the splenic architecture before and after reenlargement in three cases of failed repeated partial splenectomy after two, six and five years. Using the number of lymphoid follicles per hundred low power fields (LF/LPF) as an arbitrary index, we found that prior to the first operation 18, 20 and 27 lymphoid follicles were present per one hundred low power fields, while at the second operation, the corresponding rates were 11, 15 and 17; in control spleens, an average of 712.5 lymphoid follicles were present in one hundred low power fields. The difference in the LF/LPF ratio before and after reenlargement, led us to speculate that splenic re-enlargement in Gaucher disease is mainly the result of the continued deposition of the glucocerebroside in the reticuloendothelial system of the splenic remnant, though some degree of true regeneration as well cannot be completely ruled out. These findings are compared with animal studies and results for partial splenectomy on humans, performed for trauma. Further studies in patients with Gaucher disease are warranted to better define the underlying mechanism of splenic reenlargement.

Open lung biopsy--successful diagnostic tool with therapeutic implication in the critically ill paediatric population.

Open lung biopsy (OLB) is an important diagnostic tool in children with immune deficiency and/or chronic lung disease with diffuse pulmonary compromise. These patients require a tailored therapeutic approach owing to their fragile status and the side effects of unnecessary or inadequate treatment. Twenty-six patients of mean age 5.6 y underwent 41 open lung biopsies in our centre between 1991 and 1995. Seventeen (65%) were immunocompromised (including 13 with malignancy) and 9 had other lung diseases. The biopsies were diagnostic in 25 patients (96%), and complete clinical-pathological correlation was found in 11 (42%). A specific infectious aetiology was detected in nine patients (35%), all of them immunocompromised. Therapeutic changes were instituted on the basis of the biopsy findings in 18 patients (69%). Two patients had postoperative complications (prolonged pleural leak). We conclude that OLB is a safe diagnostic procedure even in the critically ill child and should be employed without hesitation when conventional methods fail to provide a definitive diagnosis to help redirect therapy.

A stercoraceous ulcer of the colon in neglected Hirschsprung's disease.

A large, nonspecific, chronic ulcer was found in the sigmoid colon of a 13-year-old child with neglected, undiagnosed Hirschsprung's disease (HD). There is no known association between HD and colonic ulcers, suggesting that the ulcer was a true stercoraceous ulcer of the colon and not an intrinsic defect of the aganglionotic bowel.

Should repeated partial splenectomy be attempted in patients with hematological diseases? Technical pitfalls and causes of failure in Gaucher's disease.

BACKGROUND/PURPOSE: The awareness of the risk of overwhelming sepsis after splenectomy prompted surgeons to attempt splenic preservation in patients who had hematologic diseases for which splenectomy was the conventional treatment. Partial splenectomy for Gaucher's disease was widely performed before the introduction of alglucerase. In sporadic cases a second partial splenectomy had also been attempted. METHODS: The authors present three cases of failed repeated partial splenectomy attempted before alglucerase was available. The role of angiography in planning operative strategy and the surgical pitfalls of this unusual reintervention are discussed. CONCLUSION: New indications for partial splenectomy in other hematologic diseases makes the experience gained with Gaucher's disease valuable for management decisions.

Xanthogranulomatous pyelonephritis mimicking malignant disease: is preservation of the kidney possible?

Xanthogranulomatous pyelonephritis (XGP) is an uncommon form of pyelonephritis rarely seen in children. It is characterized by destruction of the renal parenchyma and invasion of adjacent tissues, mimicking renal tumors. Preoperative diagnosis is very difficult. Two children with XGP are presented. One underwent nephrectomy and the other drainage of a renal abscess with kidney preservation. Although surgery is considered the only effective treatment, a high index of suspicion and renal biopsy may prevent radical nephrectomy.