Conjugated hyperbilirubinemia in a newborn infant after maternal (transplacental) treatment with flecainide acetate for fetal tachycardia and fetal hydrops.

An infant with intrauterine supraventricular tachycardia and fetal hydrops, successfully treated with administration of flecainide acetate to the mother had conjugated hyperbilirubinemia shortly after birth. An extensive evaluation failed to disclose a known cause. We believe that in utero exposure to flecainide acetate resulted in conjugated hyperbilirubinemia in this infant.

Fetal echocardiography: assessment of cardiovascular anatomy and function.

Echocardiography is used to assist the prenatal diagnosis group in understanding and identifying normal and abnormal structure, function, and rhythm in the fetal heart, normal variations, and change during development. Detailed segmental analysis of the heart's complex structure can be performed by experienced observers and the information used to aid in management decisions and to better understand the complex relationships of fetal cardiovascular anatomy and function, and fetal outcome. When used prudently, the technology appears to be safe to fetus and mother, but all who undergo examination will want to know of potential benefits and risks.

Leprechaunism: in vitro insulin action despite genetic insulin resistance.

We recently identified a female leprechaun infant with marked hyperinsulinemia [as high as 10,975 microU/ml (78,746 pmol/liter)], presumably secondary to insulin resistance. She had two physical findings suggestive of possible insulin action: cystic ovarian enlargement with gonadotropin-independent steroid secretion and persistent, severe myocardial hypertrophy. To examine the pathophysiology of this disorder we measured the in vitro sensitivity to insulin and other growth factors of erythroid progenitors and a T-lymphoblast cell line derived from her peripheral blood. Resistance to insulin was demonstrated by failure of her circulating erythroid progenitor cells to augment proliferation in response to physiologic concentrations of insulin (1-10 ng/ml). An immortalized T lymphoblast cell line was established by transforming the cells with the human retrovirus human T cell leukemia virus II. This cell line showed little or no response to physiologic concentrations of insulin contrary to consistently observed stimulation of colony formation by cell lines similarly derived from normals. The patient's T lymphoblasts, however, showed normal sensitivity to insulin-like growth factor I. In response to supraphysiologic insulin concentrations (25-1000 ng/ml), leprechaun T lymphoblasts showed significant augmentation of colony formation (peak 189% above baseline at 50 ng/ml); normal T lymphoblasts also showed responsiveness at these high insulin concentrations. Preincubation with a monoclonal antibody against the insulin-like growth factor I receptor (alpha IR-3 at 5000 ng/ml) blocked the in vitro effect of physiologic concentrations of insulin-like growth factor and supraphysiologic concentrations of insulin on leprechaun and control T lymphoblast colony formation, but had no clear effect upon the response to physiologic insulin concentrations.(ABSTRACT TRUNCATED AT 250 WORDS)

Treatment of fetal supraventricular tachyarrhythmias.

Supraventricular tachyarrhythmia has been encountered in 18 fetuses at the Yale-New Haven Medical Center during the past 4 years. Fourteen of these fetuses had supraventricular tachycardia and underwent in utero antiarrhythmic therapy with maternally administered digoxin either alone, or on combination with verapamil, propranolol, or procainamide. Thirteen of the 14 fetuses had successful in utero conversion of cardiac rhythm to normal sinus rhythm. The 14th patient underwent successful therapy after birth. All 14 fetuses survived despite severe fetal hydrops at the time of diagnosis in 13 of 14. The four remaining fetuses had either atrial flutter (3) or fibrillation. Two of the fetuses with atrial flutter died at birth, the 3rd survived after electrical cardioversion at birth. The fetus with atrial fibrillation converted to normal sinus rhythm and survived after maternal administration of digoxin. Using M-mode and pulsed Doppler echocardiography, the nature and electrophysiologic mechanism of the arrhythmia may be deduced. The latter information is reviewed along with the fetomaternal pharmacology of various antiarrhythmic agents to devise a rational antiarrhythmic treatment program.

Neuroblastoma with intraspinal (dumbbell) extension.

Twenty-three patients with neurogenic tumors having extradural extension (dumbbell tumors) were treated over a 30-year period. Three had ganglioneuroma, 6 had ganglioneuroblastoma, and 14 had neuroblastoma. Nineteen patients with malignant tumors had symptoms of spinal-cord compression; three patients with ganglioneuroma and one with a malignant tumor had no neurologic symptoms. Delays in diagnosis were frequent ranging from 6 weeks to 3 years in 10 patients. Sixteen patients had abnormal x-rays of the spine. All patients with malignant tumors had positive myelograms. Three patients with ganglioneuroma and 13 with malignant tumors are alive and free of disease. Seven patients with malignant tumors died: five with stage IV and 1 with stage III tumors from disease, and 1 with stage II tumor during laminectomy. Age and stage were important prognostic factors; location was not. Treatment consisted of excision and radiation or radiation only. Patients receiving lower-dose (less than 2000 rad) radiation did as well as those receiving much larger doses. Morbidity in patients with malignant tumors was high; 4 have spinal deformity (3 severe) and 8 of 13 surviving patients have residual neurologic deficits. Earlier diagnosis, a surgical approach which does not introduce morbidity, and low-dose radiation in younger patients are important factors in reducing morbidity.

Ultrasonic evaluation of the fetal human heart.

The fetal echocardiogram has demonstrated its utility for visualizing the fetal human heart during the second and third trimesters of pregnancy. The potential utility of the technique for establishing accurate anatomic diagnoses of major congenital cardiac malformations has been established. These data have been utilized to counsel prospective parents and formulate management plans for the remainder of pregnancy and the intrapartum and immediate postpartum periods. Diagnoses have been established as early as the 16th to 18th week of gestation, raising in some cases the moral and ethical question of termination of pregnancy.

Major hepatic resections for neoplasia in children.

In a consecutive series from 1968 to 1978, 11 hepatic resections encompassing at least two hepatic segments were carried out for neoplasia in pediatric patients varying in age from 7 days to 14 years. There were no operative deaths. These resections consisted of four right lobectomies, three extended right lobectomies, one right lobectomy with right nephrectomy and inferior vena caval resection, two left lobectomies, and one left extended lobectomy. Diagnoses were hepatoblastoma in six patients, Wilms' tumor invading the liver in two, hemangioendothelioma in two, and malignant mesenchymoma in one. Selective angiography and technetium Tc 99m sulfur colloid scintigraphy were important preoperative aids. Complications were infrequent and there were no major infections or biliary fistulas. There was one death eight months postoperatively due to recurrent hepatoblastoma. Vigorous hepatic regeneration occurred in all instances. Major hepatic resections are well tolerated in children and allow good subsequent development.

Operative management of stress ulcers in children.

The operative management of stress ulcer in children is controversial. Between the years 1969 and 1981, ten children were operated on at the Babies Hospital for stress ulcer. Their illnesses included connective tissue disorders (3), sepsis (2), Reye's syndrome (1), hemolytic uremic syndrome (1), leukemia (1), closed head injury (1), and renal failure (1). In those with bleeding (8), aggressive conventional medical management was attempted prior to operation. Four children also received intravenous cimetidine. Four patients underwent embolization of a feeding artery and/or selective vasopressin infusion. In those patients who perforated (2), operation was performed after a brief period of resuscitation. Ten patients underwent 11 operations. In those who bled, multiple ulcerations were the most common finding. Operative procedures consisted of partial gastrectomy and vagotomy (4), partial gastrectomy alone (2), and vagotomy and pyloroplasty (2). One child who underwent vagotomy and pyloroplasty required partial gastrectomy for recurrent bleeding. Of the two children who perforated, one was managed by plication and the other by partial gastrectomy. There were two deaths (20%), both occurring in patients who had undergone gastrectomy. One survivor has mild dumping. This experience suggests that in children (1) stress ulcers are commonly multiple when associated with major medical illnesses; (2) partial gastrectomy with or without vagotomy affords maximum protection against recurrent bleeding; (3) lesser procedures are effective for solitary bleeding duodenal ulcers or perforation; and (4) selective arterial embolization or vasopressin infusion are unreliable methods for controlling bleeding.

The suture fistula technique in the repair of selected cases of esophageal atresia.

Correction of esophageal atresia with tracheoesophageal fistula can present a difficult problem when distance and tension between upper and lower esophageal segments are too great for primary anastomosis. Alternatives have included staged procedures with upper pouch elongation or interpositions. In 1974, Shafer and David described an operation in which the lower segment was divided, closed, and then merely apposed to the unopened upper pouch. A central "necrosing suture" was placed through the lumina of both segments. Spontaneous fistulization between the two segments occurred postoperatively, and the resultant channel was then progressively dilated with woven silk bougies. This technique has been employed at Babies Hospital in five children. The results were excellent in four. One child failed to fistulize, and following a complicated postoperative course, died at home from accidental dislodgement of his tracheostomy tube. All five children required antireflux procedures but ultimately did well with regard to esophageal function.

Selective management of iatrogenic esophageal perforation in the newborn.

Since 1974 nine neonates have been treated for iatrogenic esophageal perforation. They ranged in weight from 480 to 3900 g. Four of them had been resuscitated for meconium aspiration, four were being treated for respiratory distress syndrome (RDS), and one had received only routine postpartum suctioning and gastric aspiration. In five infants, esophageal perforation was suspected following traumatic intubation of difficulty in the passage of a catheter or tube. Three neonates passed formula from chest tubes placed for pneumothorax and one child presented with persistent pneumothorax. Esophageal perforation was documented in each case by direct visualization and/or radiographic studies. Three infants were treated with antibiotics and placement of a silastic nasogastric feeding tube. An additional two infants were treated with a silastic tube, antibiotics and chest tube drainage. Four children underwent operation: gastrostomy and drainage (2), gastrostomy and closure of perforation (1), gastrostomy only (1). There were no complications of deaths. Management of iatrogenic perforation of the esophagus depends upon the extent and location of the injury. A nonoperative approach can be successful in those infants with limited injury of short duration. Operation is required in cases with extensive extravasation or delay in diagnosis.

Neonatal necrotizing enterocolitis. Survival, management, and complications: a 25-year study.

One hundred sixteen infants with neonatal necrotizing enterocolitis (NEC) have been seen during a 25-year period (1955 through 1979). Prior to 1972, the survival rate was 17%; subsequently, it has been 68%. This increased survival is attributed to (1) improved physiologic care of the critically ill infant, (2) the institution of a standard management protocol, and (3) early diagnosis and treatment. Survival following neonatal NEC is attended by a significant incidence of intestinal stricture (21%); however, physical development has been normal and there have been few long-term gastrointestinal tract problems.

Complications at menarche of urogenital sinus with associated anorectal malformations.

Persistent urogenital sinus is frequently associated with a wide spectrum of complex anatomic abnormalities involving the urinary, genital, and gastrointestinal tracts. Failure to accurately define these abnormalities can result in serious complications. One such group of complications occurs relatively late in the clinical course of these children and has received little attention. These are the complications related to menarche. This report reviews the anatomy, complications, and management in five such patients. In this group the onset of menses was associated with hydrosalpinx, pyosalpinx, hematocolpos, hematometrocolpos tubo-ovarian abscess, ruptured ovarian endometrioma, endometriosis, and peritonitis. Anatomic abnormalities included double and septate vagina, vaginal atresia and stenosis, uterus didelphys, and uterus bicornis unicollis, all predisposing to inadequate menstrual flow. Each of these children required surgical intervention. These cases stress the need for an awareness of not only the urinary but the vaginal and uterine abnormalities. Careful early definition of the anatomy and long-term follow-up of children with urogenital sinus malformation is important in order to avoid potential future complications.

Plain film detection of right aortic arch in infants with esophageal atresia and tracheoesophageal fistula.

The aortic arch can be clearly demonstrated in neonatal chest radiographs by use of a high kilovoltage-filtered film with air-gap magnification. This allows identification of the presence of right aortic arch in patients with esophageal atresia and tracheoesophageal fistula; awareness of this anomaly may influence choice of surgical approach.