The development of pyloric stenosis during transpyloric feedings.

Three infants, ages 3 to 4 months, had nasojejunal feeding tubes placed for recurrent aspiration and/or feeding intolerance after upper gastrointestinal cineradiographs (ugi) had documented gastroesophageal reflux (GER) with normal pyloric channels and prompt gastric emptying. The tubes had been in place for 3 and 4 weeks, respectively, in the first two infants when classic hypertrophic pyloric stenosis (HPS) was found during fundoplication and gastrostomy tube placement. The last child had a failed attempt at nasogastric tube feedings following 3 months of nasojejunal tube feedings. A repeat ugi suggested HPS, which was confirmed by pyloric ultrasound. This infant underwent pyloromyotomy alone. The late presentation of HPS in these infants suggests that transpyloric tubes might cause the development of HPS and exacerbate the symptoms of preexisting GER. In infants who are expected to eat by mouth, pyloromyotomy alone might allow the reinstitution of orogastric feedings without the perioperative morbidity of fundoplication and gastrostomy tube placement.

Non-immune fetal hydrops with hepatic hemangioendothelioma and Kasabach-Merritt syndrome: a case report.

A premature infant presented with non-immune hydrops fetalis, a liver mass, thrombocytopenia, and hypofibrinogenemia. Histologic examination of the liver tumor showed an infantile hemangioendothelioma. The clinical features of this case can be explained by anemia, hypoalbuminemia, and coagulopathy. The association with Kasabach-Merritt syndrome, the pathophysiology of non-immune hydrops fetalis, and primary hepatic neoplasms of the neonate are discussed.

Relationship between periventricular hemorrhage, leukomalacia and brainstem lesions in prematurely born infants.

The brain pathology in very prematurely born infants with intraventricular hemorrhage (IVH) was studied particularly as to the severity and site of the complicated brain lesions responsible for the prognosis. A high frequency of leukomalacia, pontosubicular necrosis and/or olivocerebellar neuronal loss was found in the cases of IVH, and these non-hemorrhagic brain lesions showed an increasing frequency with the grade of IVH. However, there was marked reduction of IVH, periventricular leukomalacia and, in particular, brainstem lesions in prematurely born cases of sudden infant death. These IVH and associated conditions have different pathogenesis, but factors responsible for their occurrence may be present together in each case.

Periventricular intraparenchymal cystic lesions: critical determinant of neurodevelopmental outcome in preterm infants.

During a four-year period, 154 surviving preterm infants of 32 weeks gestation or less were prospectively examined by cerebral ultrasound for periventricular-intraparenchymal cystic lesions (IPCL) subsequent to ischemic and/or haemorrhagic damage. Neurological and developmental outcome was assessed with examinations at 0, 3, 6, 12, 18, 24, 36, 48 months of age corrected for prematurity. Twenty-four (15.5%) patients were found to have IPCL changes at ultrasound. In 8 cases, a porencephalic cyst subsequent to grade IV IVH (Papile's classification) was found; all had cerebral palsy and severe developmental deficit was present in 4. Diffuse bilateral PVL was found in 8 cases: 1 was not evaluable, 7 developed cerebral palsy; the developmental delay was severe in 4, moderate in 2 patients, and only 1 was normal. Four patients had localized bilateral PVL: 3 patients had mild diplegia and 1 was normal; the developmental outcome was normal only in 1 case, 1 had a severe cognitive delay, and 2 were moderate. In the remaining 4 cases, the ultrasound showed a monolateral localized PVL: 1 patient had mild diplegia and moderate cognitive delay, 3 were normal. - This study confirms the important role of the ultrasonographic diagnosis of IPCL in preterm infants to foresee later neurodevelopmental outcome. Extensive parenchymal lesions were strongly associated with major neurodevelopmental handicaps, while localized and small lesions were correlated with more favorable neurological as well as developmental prognosis.

Early high-dose phenobarbital treatment for prevention of hypoxic-ischemic brain damage in very low birth weight infants.

In a randomized prospective trial, we studied the effect of early high-dose phenobarbital treatment on the early (intraventricular hemorrhage) and late (neurodevelopmental abnormalities) manifestations of hypoxic-ischemic encephalopathy in preterm infants weighing 1500 g or less at birth. The first intravenous dose of 15 mg/kg was given at a mean age of 110 minutes, followed by 15 mg/kg after 4 hours and then by 5 mg/kg at 24-hour intervals for 5 days. The overall incidence of intraventricular hemorrhage was 32% in treated and 46% in control infants, a nonsignificant difference. An ultrasound brain scan at 9 months old revealed no significant difference in the incidence of ventricular dilatation between treated (19%) and control (29%) infants. At 27 months, a similar incidence of mild (10%) and severe (10%) neurodevelopmental handicaps was found in both treated and control groups. Since beneficial effects could not be documented by any of the criteria used, we conclude that routine administration of phenobarbital to low birth weight infants is not justified.

Periventricular leucomalacia and neurodevelopmental outcome in preterm infants.

During an 18 month period, 120 preterm infants of 34 weeks' gestation or less were prospectively examined for periventricular leucomalacia (PVL) by cerebral ultrasound. Neurological and developmental assessment was carried out at 18 months of age corrected for prematurity in 82 surviving neonates. The developmental outcome (Griffiths development quotient) was above 80 and similar in infants with normal scans (n = 41), isolated periventricular-intraventricular haemorrhage (n = 13), and post-haemorrhagic hydrocephalus (n = 4), and no major handicap was diagnosed in these groups. By contrast, the prognosis was variable and poorer in infants with PVL (n = 24) and depended on the extent and site of the lesion. Infants with frontal PVL (n = 13) developed normally. Major sequelae (n = 8) were closely related to frontal-parietal PVL and frontal-parietal-occipital PVL and could be ascribed to the presence of cysts as well as to a persistent hyperechogenic ultrasonographic PVL appearance. A relation between size and site of the lesion and type and severity of the handicap was established.

Nissen fundoplication for gastroesophageal reflux in infants.

Some degree of gastroesophageal reflux is very common in infants and tends to reverse with time. Therefore, the indications for an antireflux operation are not well defined. Furthermore, the complication rate and the ability of the fundoplication to grow remain to be determined. To answer these questions, we reviewed the records of patients 6 months of age or younger who underwent a Nissen fundoplication with gastrostomy tube placement between 1979 and 1985. There were 45 patients (25 boys and 20 girls) with birth weights of 0.65 to 4.3 kg. The consequences of gastroesophageal reflux were more varied than in older children. Severe respiratory problems were common, including recurrent aspiration or bronchopulmonary dysplasia in 60% and frequent apneic and bradycardiac spells in 17%. Failure to gain weight was present in 20% and intractable vomiting in 2.0%. As expected, 78% of these patients had congenital anomalies or acquired problems which, in many cases, were important to the prognosis. The diagnosis was confirmed by barium swallow in all but one patient in whom gross reflux during feedings was present. Initially, medical management was tried for 3 to 4 weeks. In one patient, however, the severity of the respiratory problems precluded trial beyond 12 days. The recommendation for operation was based only on the severity of symptoms attributed to gastroesophageal reflux. All patients underwent Nissen fundoplication with gastrostomy tube placement at 2 weeks to 6 months of age and weighing 1.02 to 6.95 kg. The only surgical complication was one gastrostomy leak. Prematurity or preexisting anomalies led to a 20% incidence of late unrelated deaths between 2 weeks and 23 months postoperatively. Improvement in symptoms occurred in our survivors with follow-up of 5 to 72 months. We conclude: Significant gastroesophageal reflux in infancy most frequently produces respiratory problems that can be life threatening. Nissen fundoplication can be a safe and effective procedure in infants 6 months of age or younger. Fundoplication appears to have good growth potential, and no late complications or feeding problems have occurred. Consequently, surgical correction can be recommended for infants not responding to conservative medical therapy.

Gastroesophageal reflux and the premature infant.

Gastroesophageal reflux (GER) is a well-recognized problem in infants and children. Only scant mention of the premature infant with GER can be found in the literature. Of 760 preterm infants admitted to the NICU between 1980 and 1984, 22 had documented GER. These infants all underwent medical management including upright positioning, small frequent feeds, and often, nasojejunal feedings. Seventeen babies did not respond to medical management and underwent surgical therapy to control the reflux. Of the 17 babies requiring fundoplication, 15 had been initially intubated for treatment of respiratory distress syndrome. Eight of these 15 were extubated in less than 25 days and were improving until they exhibited sudden episodes of deteriorating pulmonary status requiring reintubation. The other seven intubated patients developed striking bronchopulmonary dysplasia (BPD) in the first month and required prolonged ventilatory support. Pulmonary deterioration, failure to grow, and refusal to eat became the herald of GER in these infants. Fundoplication dramatically improved the pulmonary status in all but one infant. Three late deaths can be attributed to cor pulmonale and pulmonary failure. BPD was striking predisposing factor for severe GER in these premature infants. In the total premature population without BDP only 8 of 684 (1.2%) had GER with five responding to medical management and three others undergoing fundoplication for apnea-bradycardia spells. Fourteen of the 76 infants with BPD (18.4%) had significant GER and all required surgical management for control of symptoms. Premature infants who develop deteriorating pulmonary function, poor growth, and/or refusal to eat should be evaluated for GER.

Periventricular leukomalacia: ultrasonic diagnosis and neurological outcome.

The ultrasonographic findings and neurological development of 15 preterm infants, born between August 1981 and July 1984, who developed periventricular leukomalacia (P.V.L.) are described. The incidence of P.V.L. in infants with a birth weight less than 1 500 g was 2.3%. Nine of the 15 infants demonstrated areas of increased echogenicity in the periventricular regions prior to the development of cystic changes. The mean age for the appearance of cysts was 26 days. At follow-up 14 infants showed evidence of abnormal motor development and 8 infants had visual defects. All infants appear to have normal hearing, but 3 infants show signs of delayed speech. To date, only 2 infants demonstrate signs of mental retardation, but many of the infants are too young to assess intelligence accurately.

Infantile esotropia: results in the neurologic impaired and "normal" child at NCMH (six years).

Charts of all infantile esotropes who received their primary surgical procedure at the North Carolina Memorial Hospital, between February 1978 and June 1984 were reviewed. Neurologic problems (general and ocular) were identified in 29 of the 47 patients (61.7%) followed a minimum three months (mean = 25.0 +/- 2.9 mos). Frequent general neurologic problems were prematurity, hydrocephalus, mental retardation, cerebral palsy, meningomyelocele, intraventricular hemorrhage, and seizures (neonatal and/or postnatal). Abducens nerve palsy was the most common ocular neurologic impairment. Neurologic impaired esotropes were older (mean = 31.9 +/- 3.8 mos) than the "normal" group (mean = 17.0 +/- 3.3 mos) at the time of surgery. Recession/resection procedures were performed on 13 (44.8%) of the neurologically impaired and nine (50.0%) of the normal esotropes. Bimedial recessions were employed on 12 (41.4%) of the patients with neurologic problems and seven (38.8%) of the normal esotropes. Unilateral medial recessions and/or inferior oblique recessions were performed on six patients. At last follow-up, orthophoria (+/- 10 delta) was present in 16 (55.2%) neurologically impaired patients and 15 (83.3%) normal esotropes (p less than 0.05 by the normal deviate (z) test). Seven (24.1%) neurologically impaired patients had residual esotropia, while consecutive exotropia was present in six (20.7%) patients. Among normal esotropes, residual esotropia was found in one patient and consecutive exotropia in two (11.1%) patients. DVD's occurred in nine patients while four subjects developed a postoperative accommodative component.

Small tube peritoneovenous shunting for the management of neonatal intractable ascites.

A ventriculoperitoneal shunt for infants has been successfully adapted to function in a reversed manner by sending ascitic fluid back into the circulation of the premature infant. This technique permits successful peritoneovenous shunting in the smallest infant.

Inguinal hernias after ventriculoperitoneal shunt procedures in pediatric patients.

The results of this study of 134 patients confirm the increased incidence of previously unrecognized inguinal hernias after ventriculoperitoneal shunting procedures. Infants with intraventricular hemorrhage appeared to be the most susceptible, although associated prematurity may play an additional role. Close observation of infants and children who undergo ventriculoperitoneal shunting is required, to allow early detection and repair of inguinal hernias. Inguinal herniorrhaphy can be performed safely with few or no postoperative complications and no deleterious effects to the VP shunt.

Clinical course to 1 year of age in premature infants with patent ductus arteriosus: results of a multicenter randomized trial of indomethacin.

Reported are 1-year follow-up results of a randomized clinical trial comparing three strategies of managing clinically significant patent ductus arteriosus at the time of diagnosis in premature infants: (1) immediate administration of a three-dose course of intravenously administered indomethacin in addition to usual medical therapy (fluid restriction and use of diuretics or digitalis or both), with surgery as a backup measure, (2) usual medical therapy alone initially, with indomethacin as the first and surgery as the final backup measure, and (3) usual medical therapy alone initially, with surgery alone as backup. Of primary concern were the relative merits of these three managements strategies in the terms of the long-term occurrence of a wide range of health problems. Although at the time of neonatal hospitalization there was a significant excess of bleeding episodes in infants receiving indomethacin as part of initial treatment, and a significantly higher rate of retrolental fibroplasia in the those given usual medical therapy with surgery as backup, there were no statistically significant differences at 1 year of age related to these intermediate outcomes. In other regards, too, the treatment strategies appeared interchangeable in terms of the 1-year outcome.

Surgical closure of patent ductus arteriosus in 268 preterm infants.

Over a 2 year period ending in April, 1981, 268 premature infants with birth weight below 1,750 gm underwent operation for a "hemodynamically significant" patent ductus arteriosus. Operations were performed in 13 centers participating in a collaborative study, which was primarily designed to evaluate the role of indomethacin in the management of patent ductus arteriosus. No patient died during the operations, which were done at a median age of 10 days. Eight infants (3%) died within 36 hours after operation. In only one was the death directly attributable to the operative procedure. Hospital mortality (23%) and postoperative morbidity, which included bronchopulmonary dysplasia, pneumothorax, and sepsis, were unrelated to birth weight, age at operation, and degree of preexisting pulmonary disease or preoperative treatment of the infant with indomethacin. Results indicate that surgical ligation is a safe and effective procedure for treating patent ductus arteriosus with large left-to-right shunting in small premature infants.