Posterior mediastinal masses.

Solid mediastinal masses in infancy and childhood occur most frequently in the posterior mediastinum. From 1972 to 1989, 63 patients presented with a posterior mediastinal mass. The median age at diagnosis was 6 years (range, 1 day to 26 years). Thirty patients were female. Forty-five percent of the patients presented with respiratory symptoms or chest pain; 13% had neurologic symptoms, one half of which were related to spinal cord compression; and 5% had a palpable mass. In 32% of patients the mass was an incidental finding. The tumors were of neurogenic origin in 89% of patients, of which neuroblastoma was the most common. Of all patients with posterior mediastinal masses, 60% had malignant tumors. Median follow-up for 62 of 63 evaluable patients was 45 months (range, 1 to 289 months). One patient was lost to follow-up. Of the 62 patients followed, 84% are alive and free of disease. All but 4 of the 32 patients with neuroblastoma are alive and free of disease with a median follow-up of 73 months (range, 7 to 289 months). Patients with neuroblastoma who were diagnosed in the first year of life had a significantly better survival pattern than those presenting after the first year. There were seven deaths in the series: four from neuroblastoma, two from primitive neuroectodermal tumor, and one from malignant schwannoma. Preoperative diagnostic evaluation of a posterior mediastinal mass should include posteroanterior and lateral chest roentgenograms, and either CT or MRI of the chest and abdomen to assess the extent of the mass.(ABSTRACT TRUNCATED AT 250 WORDS)

Outcome of renal transplantation in children less than two years of age.

Twenty-two renal transplants were performed in 21 children less than two years of age at Children's Hospital. Fourteen were from living related donors and eight were from cadaveric donors. The five year patient and graft survivals of these recipients were compared to all other pediatric recipients between two and 18 years of age who received renal transplants over the same time period. Five year graft survival for recipients less than two years of age was 86% following living-related donor transplantation and 38% following cadaver donor transplantation. Older pediatric recipients aged between two and 18 years had a five year graft survival of 73% following living-related donor renal transplantation, which was similar to that for recipients less than two years of age. Although older cadaveric recipients had a comparable five year graft survival to younger recipients, at 42%, the patterns of graft loss were different. Graft failures in young recipients occurred within the first seven months post-transplant, whereas the older recipient's grafts failed more gradually. Actuarial five-year patient survival in recipients less than two years of age was 86% following living-related donor renal transplantation and 70% following cadaver-donor renal transplantation. Recipients less than two years of age had a poorer patient survival than older recipients following both living-related donor renal transplantation (P = 0.06) and cadaver-donor renal transplantation (P less than 0.05). These findings suggest that the graft survival of living-related donor renal transplantation in recipients less than two years of age is better than that of cadaver-donor renal transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)

Retroperitoneal alimentary tract duplications detected in utero.

The prenatal diagnosis of numerous congenital anomalies has become routine. The prenatal diagnosis of cystic lesions of the retroperitoneum can be due to a variety of renal, gastrointestinal, or adrenal lesions. This finding demands aggressive postnatal follow-up to rule out the possibility of cystic adrenal neuroblastoma. We report the first cases of retroperitoneal cystic masses diagnosed in utero that ultimately proved to be enteric duplications. Therefore, the differential diagnosis of cystic masses of the retroperitoneum found prenatally should be expanded to include enteric duplication cysts.

Rectal duplications.

Recent experience with two cases of rectal duplication, which had been misdiagnosed as hemorrhoids, or fistula-in-ano with resultant delay in diagnosis, prompted us to review our prior experience with 11 of these unusual cases. Age at presentation ranged from newborn to 18 years (mean, 17 months). The most common presenting sign was a perianal or anal fistula, observed in five children. Two children presenting with fistulae had concomitant infection in the duplication. Other presenting signs included obstruction or prolapse caused by the rectal mass in three patients, rectal bleeding in three, and urinary retention in one. Some children presented with more than one finding. No associated spinal or vertebral anomalies were observed. Total excision was performed using a transanal approach in eight patients, postanal (transcoccygeal) in two, and posterior sagittal in one. Postoperative continence was normal in all patients. These cases illustrate that rectal duplications can be confused with other types of anorectal pathology including hemorrhoids, fistula-in-ano, and perirectal abscess. Total excision performed using a posterior sagittal, transanal, or transcoccygeal approach is curative.

The therapy of biliary atresia combining the Kasai portoenterostomy with liver transplantation: a single center experience.

Survival has improved dramatically for children with extrahepatic biliary atresia (EHBA), but optimal surgical management remains controversial. We have studied 28 infants born between June 1981, and April 1988, who underwent Kasai's portoenterostomy as primary surgical treatment. Those with evidence of subsequent hepatic decompensation were evaluated for liver transplantation (LT). All were cared for by surgeons who perform both the Kasai portoenterostomy and LT. Following the Kasai operation, 16 of 28 patients (57.1%) have achieved total biliary drainage, while 5 of 28 (17.9%) achieved partial drainage, and 7 of 28 (25%) achieved no drainage. Nine of 28 (32.1%) have undergone LT, 4 of whom were transplanted at greater than 2 years of age. To date, 25 of 28 (89.3%) are alive. Twenty three of 25 (92%) living are jaundice-free, 65% of whom have undergone the Kasai operation only. We project that 5 of 28 (17.8%) will come to transplantation, and 12 of 28 (43%) have no current indication that they are likely to come to transplantation. We conclude that combining Kasai's portoenterostomy with LT as needed is an effective therapy for children with EHBA, that the Kasai operation offers significant benefit by delaying LT in many, and that the long-term results of management remain to be determined.

Fate of the distal esophageal remnant following esophageal replacement.

When a colon segment is used for esophageal replacement in patients with esophageal atresia, the distal esophageal remnant is often left in place. We recently treated two patients who developed esophagitis in their esophageal remnants and did not respond to cimetidine and antacids. They were treated by esophagectomy at 22 and 37 years of age with relief of symptoms. One had an ulcer and stricture near the gastroesophageal junction and the second had gastric mucosa (Barrett's esophagus) replacing most of the squamous epithelium. Six additional patients were then reviewed who had resection of their distal esophageal segments between 1978 and 1987. Esophagitis was present in all. Also identified were two specimens with Barrett's esophagus and four with mural bronchial glands as well as surface respiratory and metaplastic squamous epithelium in two, and cartilagenous remnants in two. The findings of chronic inflammation in the esophageal remnant and three cases of Barrett's esophagus raise concern about the possible long-term complication of malignancy. Therefore, we recommend that esophagectomy be performed at the time of esophageal replacement if feasible, or later if symptoms occur or barium studies show esophagitis or ulceration.

Bilateral neonatal torsion.

Extravaginal neonatal torsion of the spermatic cord, although rare, is being recognized with increasing frequency. Bilateral neonatal torsion is even more uncommon. Only 1 previous case of asynchronous neonatal torsion has been reported. Controversy exists concerning the urgency of exploration as well as the need for contralateral orchiopexy, since some reports suggest that the contralateral testicle is not at risk for torsion. We present 4 consecutive cases of bilateral neonatal torsion seen between 1966 and 1986. Two cases were asynchronous, 1 of which with a previously normal-appearing contralateral gonad was rendered anorchid. In the other patient a testicle was salvaged by prompt intervention. We have adopted a policy of prompt exploration with contralateral orchiopexy in all cases of unilateral neonatal torsion. Treatment of the ipsilateral testicle is determined by operative findings.

Juxtapancreatic intestinal duplications with pancreatic ductal communication: a cause of pancreatitis and recurrent abdominal pain in childhood.

Pancreatic duplications with ductal communications should be included in the differential diagnosis of any child presenting with recurrent abdominal pain of unknown etiology and should be considered as a possible cause of pancreatitis in childhood. Such duplications most likely arise from nonregressing diverticula of the pancreatic bud during embryologic development. Their clinical presentation is unique from other duplications because of their anatomic association with the pancreatic duct. Pain and weight loss are the major presenting complaints, although many patients have nausea and vomiting. Serum chemistries, in particular the serum amylase, are usually normal and are of little help in the differential diagnosis. Radiographic evaluation has not been particularly helpful in the past. ERCP, ultrasonic examination, and CT scan show great promise, however. Operative intervention should be tailored for the individual patient. The operation performed will depend upon operative findings. Intraoperative pancreatograms or cystograms are very helpful in differentiating these cysts from others at the time of operation. Pathologically, most of the duplications have a thickened muscular coat that usually has some evidence of inflammation. They are usually lined with gastric mucosa. Except in the most severe cases, the pancreas is histologically normal, suggesting that most of the pain experienced by these patients is secondary to inflammation within the duplication. The inflammatory response may completely destroy the mucosal lining and cause fibrosis within the muscular coat of the duplication. In those instances, these lesions cannot be differentiated from pancreatic pseudocyst. This may account for some of the "idiopathic" pseudocysts reported in the literature.

Tracheal bronchus: association with respiratory morbidity in childhood.

An aberrant right upper lobe (RUL) bronchus arising from the trachea (tracheal bronchus) can be responsible for recurrent pneumonia. In this hospital, 2% of children requiring bronchoscopy for respiratory symptoms are found to have a tracheal bronchus, which is frequently thought to be an incidental finding. We reviewed findings in 18 patients to determine when a tracheal bronchus is of clinical significance. The age at presentation ranged from 1 day to 54 months (mean 17 months). The children had recurrent pneumonia (nine), stridor (six), respiratory distress (two) and a thoracic mass (one). Other congenital abnormalities were present in 14, including Down syndrome (two), tracheoesophageal fistula (two), and fused or hypoplastic first and second ribs (four). Recurrent RUL pneumonia was present in five. Bronchiectasis or bronchial stenosis was shown by bronchography in four of five; in all five the right upper lobe was surgically resected, with resolution of the recurrent pneumonias. The presence of a clinically significant tracheal bronchus should be considered in every child with recurrent RUL pneumonia, especially in children with Down syndrome or rib abnormalities; if bronchiectasis or bronchial stenosis is found, surgical resection should be performed.

Pancreatitis and intravenous fat: an association in patients with inflammatory bowel disease.

Since recently adding Intralipid (IL) to our standard total parenteral nutrition (TPN) regimen for children with inflammatory bowel (IBD) disease, two children undergoing major intestinal resections have developed acute pancreatitis in the early postoperative period. Herein, we present the clinical summaries of these two patients, discuss the multifactoral etiologies of acute pancreatitis, and review the role of emulsified fat solutions in TPN in children with IBD.

Primary bronchial tumors in childhood. A clinicopathologic study of six cases.

A primary bronchial tumor was diagnosed in six children younger than age 12 years. These cases include four bronchial carcinoids, a low-grade mucoepidermoid carcinoma, and a granular cell tumor ("myoblastoma"). Lobar or segmental resection was performed in each case and remains the treatment of choice. The most common bronchial tumors in childhood are carcinoids with 17 other evaluable cases in the English Literature. This series includes the youngest child to be reported with bilateral choroidal metastases leading to blindness; in addition, there were clinical manifestations of gigantism and acromegaly possibly related to ectopic hormone production. Low-grade mucoepidermoid carcinomas rank second in frequency to carcinoids and have an excellent prognosis. Judging from the childhood bronchial tumors reported to date, proved examples of bronchogenic carcinoma are vanishingly rare.

Course and prognosis after colectomy and ileostomy for inflammatory bowel disease in childhood and adolescence.

The clinical course of 32 pediatric patients with inflammatory bowel disease (ulcerative colitis--18, Crohn's disease--14) after colectomy and ileostomy was investigated. Specific indications for surgery were: ulcerative colitis (severe colitis--50%, chronic disease--50%); Crohn's disease (fistulae--28%, severe colitis--28%, chronic disease--21%, hemorrhage--14%, growth failure--7%). Proctectomy was performed at the time of colectomy in 72% of patients with ulcerative colitis and in 64% of those with Crohn's disease. Major post-operative complications (stomal dysfunction without documented recurrent disease, obstruction, abscess, bleeding) were observed in 60% of all patients regardless of diagnosis. Histologically documented recurrent disease developed in 42% of the patients with Crohn's disease (mean follow-up: 5 yr) despite removal of all affected bowel. Ileal disease at the time of surgery appeared to be a poor prognostic factor. Sexual activity was either improved or unchanged in sexually active patients. Impotence was not observed in any of the male patients. Despite complications and recurrent disease, the majority (75%) of patients viewed colectomy and ileostomy as improving the quality of their life.

Mortality of chronic hemodialysis and renal transplantation in pediatric end-stage renal disease.

The long-term mortality of chronic hemodialysis and renal transplantation was analyzed in all children treated for end-stage renal disease at Children's Hospital Medical Center over the pase 8 1/2 years. A total of 216 transplantation or dialysis courses in 120 patients were studied. No patients were excluded from treatment or analysis. Overall actuarial survival was 92% at six months, 90% at 12 months, and 89% at five years. When actuarial survival for each form of treatment was examined, patient survival was 100% at six months and 95% at five years for chronic hemodialysis; 92% at six months and five years for living related transplantation; and 88% at six months and 85% at five years for cadaveric transplantation. We conclude that most children with end-stage renal disease can be kept alive with current treatment programs, and that the mortality of chronic hemodialysis in children is comparable to that of renal transplantation.

Polyalveolar lobe: anatomic and physiologic parameters and their relationship to congenital lobar emphysema.

Polyalveolar lobe is one of the recently described pathological entities that can give rise to congenital lobar emphysema (CLE). In polyalveolar lobe, the total alveolar number as determined by microscopic point-counting of randomly taken lung sections is increased three to fivefold. The airways and arteries are normal for age in number, size and structure. Follow-up over 10-20 yr of patients operated upon for congenital lobar emphysema, including those of polyalveolar lobe etiology shows that these patients do well clinically. All patients have some pulmonary function abnormalities, specifically a decrease in airway conductance, and forced expiratory volume. It is not possible to distinguish patients with polyalveolar lobes from other CLE patients on the basis of pulmonary function data. All patients had normal lung volumes and vital capacities, despite losing 8%-20% of their pulmonary parenchyma. Based on compensatory lung growth, it may be preferable to operate earlier, rather than later in these cases.

Achalasia in childhood: a 20-year experience.

The incidence of achalasia in children is lower than that in adults, but the disturbances in growth and development, and the severity of pulmonary symptoms are more profound. We have treated 20 children with the disease over the past 20 yr. All children were first treated with dilatation of the cardioesophageal junction either with filoform and followers, or in older children, pneumatic dilatation under fluoroscopic control. Five children, all older than 9 yr of age and all female, had prolonged relief of symptoms following two dilatations and never required a surgical procedure. Fifteen children responded unsatisfactorily to repeated dilatation and 12 of these accepted a surgical procedure. A modifier Heller procedure was performed in 11 children and one patient was treated with a transthoracic cardioplasty. The operations resulted in dramatic relief of symptoms, satisfactory weight gain, and the disappearance of pulmonary symptoms in every case. We recommend that all children under 9 yr of age should have a modified Heller procedure as primary therapy. In children older than 9 yr of age, dilatation is an appropriate initial therapy but should not be repeated unless significant improvement results.

Congenital lymphedema of the penis: a method of reconstruction.

Congenital lymphedema of the genitalia has profound physical and psychological consequences for the growing child. Extensive resection of this tissue and reconstruction by skin grafting offers a less than satisfactory cosmetic result. Over the past year we have employed a method of total excision of the lymphedematous tissue of the penile shaft with cosmetic reconstruction without skin grafting. A circumferential incision was made 5-10 mm from the coronal sulcus and deepened to the level of Buck's fascia. The skin and subcutaneous tissue were then completely dissected away from the penis. The skin was everted and all of the abnormal lymphedematous tissue excised up to the dermal skin margin. The skin was then tailored to the size of the penile shaft and reapproximated. This method has been employed in two patients with the advantages of (1) shorter hospitalization, (2) lack of morbidity associated with the skin donor site, and (3) satisfactory cosmetic results.