Eduardo T. Fernandes

Neurenteric cyst: Surgery and diagnostic imaging

Neurenteric cysts are rare, with fewer than 30 cases noted in the literature. We report the case of a newborn infant with respiratory distress caused by a large neurenteric cyst that was identified by prenatal ultrasound. Treatment consisted of excision of the mass through a right posterolateral thoracotomy. The cyst adhered to the spine at the level of the first thoracic vertebra and communicated with the jejunum through a posterior diaphragmatic defect. Postoperative studies with magnetic resonance imaging (MRI) and computed tomography (CT) disclosed an anterior meningocele and tethering of the spinal column. This is the second reported case of a neurenteric cyst demonstrated by prenatal ultrasound. The presence of an intrathoracic cyst associated with spinal abnormalities is characteristic of this anomaly. With imaging techniques such as MRI and CT, we may detect residual intraspinal disease associated with neurenteric cysts.

Teratoid Wilms' Tumor: The St Jude Experience

The term teratoid Wilms tumor has been used recently to describe an unusual type of tumor in which, although classic nephroblastoma tissue is present, there is a significant diversity of cell types and tissues present. Examination of tissue samples from 290 patients treated at St Jude Childrens Research Hospital from 1964 to 1987 disclosed that three children had teratoid Wilms tumor. All three children had renal tumors and two of them presented with bilateral pyeloureteral obstruction, uremia, and hypertension. When compared with classic nephroblastoma, two of the children with teratoid elements responded poorly to chemotherapy and irradiation, although no metastatic disease was identified. One child died with sepsis and renal failure; the other two are surviving disease-free for 7 1/2 years and 26+ months since diagnosis. Because of the tendency for bilateral involvement, ureteral obstruction, and uremia, and their relative resistance to chemotherapy and irradiation, surgery is the principal form of therapy for patients with these tumors.

Extrarenal Wilms' tumor

The extrarenal location of Wilms tumor is extremely rare. These tumors can arise from other neoplasms, generally teratomas, or they can present without any associated teratomatous elements. We encountered only 19 well-documented cases of isolated extrarenal nephroblastoma, and we describe three previously unreported patients with this disease. Two of the three children presented also with horseshoe kidneys, an association that may have clinical and embryologic significance. The presence of tumor cephalad to a horseshoe kidney is easier to explain when we consider that they originate from primitive mesodermal tissue, probably mesonephric rests, and not from metanephric remnants. Also, this association should alert physicians to the possible diagnosis of extrarenal nephroblastoma in patients with a retroperitoneal mass and horseshoe kidneys. These patients should be treated according to the same protocols as those for patients with intrarenal Wilms tumor.

Preduodenal portal vein: Surgery and radiographic appearance

Preduodenal portal vein is rare, with 63 cases reported in the literature. In general, this anomaly occurs in children with associated small bowel obstruction. We report a newborn infant who presented with duodenal stenosis, mongolism, and preduodenal portal vein. Treatment consisted of a duodenoduodenal anastomosis without mobilizing the portal vein. The correlation between imaging techniques and the operative findings is discussed. Because identification of preduodenal portal vein at surgery is important, preoperative sonography may be useful in selected cases to define the position of the vein.

Two decades of experience with testicular tumors in children at St Jude Children's Research Hospital

From 1968 to 1988, 24 children and adolescents with malignant testicular tumors were treated at St Jude Childrens Research Hospital. Pure yolk sac tumors (YST) were present in 13 cases; 11 patients had other types of nonseminomatous malignant germ cell tumors. Children with localized and totally resectable disease (stage I) were treated by orchiectomy alone; all others also received chemotherapy. Five of ten patients treated before the implementation of a multiagent chemotherapy protocol in 1979 have died. By contrast, all of the 14 patients treated on this protocol are alive. The improved survival during the past decade is attributable to better diagnostic imaging techniques, the availability of serum tumor markers to monitor disease activity, and more effective chemotherapy. Orchiectomy alone is sufficient treatment for patients with clinical stage I disease who show appropriate reductions in tumor marker levels after surgery. Modern platinum-based chemotherapy provides disease control in patients with higher stage disease.

Extralobar pulmonary sequestration and mediastinal bronchogenic cyst.

A child with coexisting mediastinal bronchogenic cyst and extralobar pulmonary sequestration is presented. Two distinct lesions were suggested by barium esophagram and confirmed by a chest computed tomography scan. The diagnostic and embryologic relationship of the two lesions is discussed.

Mediastinal mass and radiolucent esophageal foreign body

The diagnosis of radiolucent esophageal foreign bodies can be difficult, particularly in patients with predominant respiratory symptoms. The consequences of the impaction of a foreign body in the esophagus are serious, and esophageal stenosis, perforation, acquired tracheoesophageal fistulas are among the complications already reported. An unusual complication of a nondiagnosed radiolucent plastic coin that remained impacted for 11 months on the posterior esophageal wall of a 20-month-old child, who presented only with respiratory symptoms, is reported. The foreign body eroded through the esophageal wall, causing an intramural abscess that was initially interpreted as a mediastinal mass, and the patient was operated on with the diagnosis of a foregut duplication. Literature on this situation was reviewed, and the problems associated with the diagnosis and treatment of children with radiolucent esophageal foreign bodies are discussed.

Management of the brown recluse spider bite

The objectives in the treatment of the brown recluse spider bite are to prevent skin necrosis and the need for reconstructive surgery. A simple technique that consists of curetting the subcutaneous tissue in the necrotic area of the lesion, to prevent the local destructive actions of the toxin, is described. From 1981 to 1987, 18 patients were treated with this technique. The wounds were generally curetted under local anesthesia in an outpatient setting. In all but one case, the erythema, edema, and pain resolved significantly in 24 to 48 hours, and the wounds healed primarily with minimal scarring. We consider curettage the treatment of choice for brown recluse spider bites when the lesions are noted in relatively early stages. It can be performed as an outpatient procedure; it controls the symptoms and prevents further necrosis, with excellent cosmetic results.

Preventing accidental decannulations following tracheostomy

Tracheostomy in infants and children is associated with a high rate of accidental decannulation in the early postoperative period. Eighty-eight patients underwent tracheostomy from 1980 to 1985, and 22 were sutured in place. Accidental decannulation occurred in 31.8% of the nonsutured and 4.5% of the sutured tracheostomies. We advocate suturing tracheostomy tubes in place in infants and children and describe a simple suture technique for this purpose.

Urinary Nitrogen Constituents in the Postsurgical Preterm Neonate Receiving Parenteral Nutrition

Minimal information is available defining urinary nitrogen constituents in preterm neonates receiving parenteral nutrition (PN). The study objective was to evaluate 24-hour urine collections for total urinary nitrogen (TUN), urinary urea nitrogen (UUN), and the nitrogen content in creatinine, ammonia, free amino acids, protein, hippuric acid, and uric acid at baseline (days 1 to 2 of PN and days 1 to 3 after surgery) and 7 days later in eight preterm, postsurgical neonates. Calculation of undetermined nitrogen was also completed. Comparisons with historic, normal data were made for each urinary nitrogen constituent. At baseline, PN provided 59 +/- 10 nonprotein kcal/kg.day-1 and 430 +/- 54 mg/kg.day-1. At day 7, PN provided 106 +/- 23 nonprotein kcal/kg.day-1 and 432 +/- 30 mg/kg.day-1. TUN, UUN, and protein nitrogen decreased significantly from baseline at day 7 (p < .05). The percentages of TUN as amino acids, creatinine, and uric acid nitrogen were calculated. Percent amino acid nitrogen (6.0 +/- 2.3% vs 8.4 +/- 1.5%, p < .05), percent creatinine nitrogen (1.6 +/- 0.5% vs 2.9 +/- 0.8%, p < .001) and percent uric acid nitrogen (1.7 +/- 0.9% vs 3.6 +/- 2.1%, p < .05) increased significantly at day 7. The observed urinary free amino acid nitrogen fraction represented a higher percentage of TUN both at baseline and at day 7 when compared with term neonatal reference data, whereas creatinine nitrogen, uric acid nitrogen, and protein nitrogen represented a lower percentage of TUN. However, amino acid and creatinine nitrogen as a percentage of TUN were similar to levels in milk formula-fed preterm infants.(ABSTRACT TRUNCATED AT 250 WORDS)