Meconium obstruction in markedly premature infant.

Markedly premature infants may present with intestinal obstruction and perforation secondary to inspissated meconium in the absence of cystic fibrosis. Between 1990 and 1994, 13 patients were treated for intestinal obstruction secondary to inspissated meconium. The average birth weight was 760 g. Prenatal and postnatal risk factors were identified, and included intrauterine growth retardation, maternal hypertension, prolonged administration of tocolytics, patent ductus arteriosus, hyaline membrane disease, and intraventricular hemorrhage. Stooling was absent or infrequent during the first 2 weeks of life. Surgical presentation consisted of distension and/or perforation between days 2 and 17 of life. Twelve patients required operative intervention. Findings invariably included one or more obstructing meconium plugs with proximal distension and frequent necrosis of the dilated segments. Surgical options consisted of resection or enterotomy, accompanied by primary closure or by distal irrigation and exteriorization. Irrigation led to iatrogenic bowel injury in two patients. One patient was managed successfully with oral and rectal gastrograffin and oral acetylcysteine. Ten patients were discharged, all of whom had normal stooling patterns and tested negatively for cystic fibrosis. Three patients died, two from the primary disease. The markedly premature infant is at risk for obstruction and eventual perforation secondary to meconium plugs, presumably formed in conjunction with intestinal dysmotility. Prompt diagnosis and timely intervention require a high index of suspicion, attention to stooling patterns and abdominal examinations, and screening radiographs when indicated.

Outpatient inguinal herniorrhaphy in premature infants: is it safe?

Because postoperative apnea and bradycardia in premature infants following inguinal herniorrhaphy remains a concern, outpatient repair has not been recommended. We have been performing outpatient inguinal herniorrhaphy in premature infants and the present study reviews our experience. Between 1985 and 1990, 1,294 outpatient inguinal herniorrhaphies were performed. Of this group 124 patients (9.6%) were identified as being premature (less than or equal to 36 weeks gestational age). Average ages were: gestational age 32.7 weeks (range, 24 to 36 weeks); postnatal age 12.6 weeks (range, 3 to 24 weeks); and postconceptional age (gestational plus postnatal) 45.3 weeks (range, 34 to 59 weeks). Twenty-two infants previously required ventilatory support, 11 patients had apnea/bradycardia, and 9 patients developed bronchopulmonary dysplasia. General anesthesia (usually nitrous oxide and fluothane) was used in all patients and 75% underwent endotracheal intubation. The average operating room time was 40 minutes (range, 20 to 115 minutes) and the average recovery room time was 94 minutes (range, 30 to 240 minutes). There were no perioperative deaths. One patient became apneic immediately after extubation in the operating room. No further episodes were noted after 4 hours of observation. Another patient following discharge had a brief apneic episode at home while on an apnea monitor, which was relieved with gentle stimulation. Both patients had no further sequelae. Bradycardia to 80 beats/min was noted in two patients, and resolved spontaneously in the recovery room. Laryngospasm after extubation in the operating room occurred in two patients, one of whom required brief reintubation and the other resolved spontaneously. Two patients required postoperative ventilation: one was extubated in the recovery room and the other was hospitalized for 24 hours.(ABSTRACT TRUNCATED AT 250 WORDS)

Familial abdominal wall defects.

We report 2 families, each having multiple sibs with abdominal wall defects. In family 1, normal parents gave birth to identical (monochorionic, diamniotic) twins. This is the first reported case of gastroschisis occurring in monozygotic twins. In family 2, a normal mother gave birth to a son with omphalocele. Two subsequent pregnancies with a different husband resulted in a stillborn girl with partial atresia of the colon and a liveborn girl with gastroschisis. In neither case were there any associated anomalies. In neither of the 2 families was there consanguinity or history of other abdominal wall defects. The familial occurrence of these defects suggests that 1) multifactorial determination should be considered in at least some cases of abdominal wall defects, 2) the bowel atresias that occasionally accompany gastroschisis may also have a genetic (multifactorial) cause, and 3) some cases of gastroschisis and omphalocele may have the same genetic cause.

Primary repair without routine gastrostomy is the treatment of choice for neonates with esophageal atresia and tracheoesophageal fistula.

Gastrostomy and staged repair are techniques frequently recommended for the management of esophageal atresia with distal tracheoesophageal fistula (EA-TEF), especially for those infants at high risk. We describe 42 consecutive patients with EA-TEF treated during the past 8 years. Staged repair and preliminary gastrostomy were not routinely employed. Fifteen infants were considered to be at high risk (Waterston class C). Surgical treatment via an extrapleural approach consisted of fistula division and primary single-layer end-to-end esophageal anastomosis. Four patients required proximal esophageal circular myotomy. Four patients early in the series received a gastrostomy at or before definitive repair for various life-threatening indications. One patient had fistula division only and died before esophageal anastomosis was possible. Two neonates died before repair and another died after repair. The deaths in this series of patients were unrelated to EA-TEF. One patient developed a clinically significant anastomotic leak. Four patients required multiple dilatations for anastomotic stricture. Fundoplication was necessary in 3 patients with symptomatic gastroesophageal reflux. Our data demonstrate that excellent overall survival (90%) with low morbidity (15%) can be achieved using primary repair without preliminary gastrostomy in most neonates with EA-TEF. We believe that mortality in high-risk patients with EA-TEF is due to associated life-threatening anomalies.

Complications of prematurity that may require surgical intervention.

Many complications related to prematurity may require surgical intervention. Between July 1981 and July 1987, treatment of patent ductus arteriosus (PDA) (228 patients), necrotizing enterocolitis (NEC) (49 patients), and complications of high-pressure ventilation (eight patients) was reviewed. A PDA was ligated in 136 patients, with one death and one complication. Ninety-two patients had treatment with indomethacin, with 35 failures. A PDA was associated with NEC in 37 of the 49 patients, with a 73% mortality when they occurred within 72 hours of each other. Two patients died following pulmonary resection for lung cysts. The two patients with pneumoperitoneum and pneumopericardium were successfully treated with tube drainage. A PDA ligation was successful, with low mortality and morbidity. Treatment with indomethacin was unsuccessful in 38% of patients. There is a high mortality when NEC and PDA occur within 72 hours of each other.

Association of closure of patent ductus arteriosus and development of necrotizing enterocolitis.

Over a 5-year period ending in June 1986, 234 neonates with evidence of a significant patent ductus arteriosus (PDA) underwent ductal manipulation. Thirty-four infants (15%) developed evidence of necrotizing enterocolitis (NEC). When NEC and treatment of PDA were within 72 hours of each other, there was a 71% mortality rate. When NEC and PDA ligation were greater than 72 hours apart, there were no deaths. Development of NEC prior to ductal closure was associated with a mortality of 57%, as opposed to no mortality when the development of NEC occurred after ductal closure. Our data suggest that infants who develop NEC before PDA ligation incur a high mortality.

Management of patent ductus arteriosus: a comparison of operative v pharmacologic treatment.

Over a 5-year period ending June 1986, 183 premature infants with evidence of a hemodynamically significant patent ductus arteriosus (PDA) associated with cardiopulmonary compromise underwent pharmacologic and/or surgical ductal manipulation. One hundred seven infants underwent surgical ligation and 76 initially received indomethacin. The average birth weight was 10% less and 1 week less for the surgically treated v the indomethacin-treated infants. Among the infants undergoing ligation, there were no failures of therapy and one surgically related complication. Among the infants receiving indomethacin, 42% failed to improve, and 84% of these infants required surgical intervention. Those infants who failed indomethacin therapy in general weighed less, had a shorter gestation and required prolonged ventilatory support. In no instance was death directly attributable to either therapeutic modality. Our data suggest that surgical ligation of hemodynamically significant PDA yields a more predictable result with low morbidity and no mortality. We believe it is the preferred treatment for premature infants less than 800 g.

"Balanced" thoracic drainage is the method of choice to control intrathoracic pressure following repair of diaphragmatic hernia.

Respiratory failure from pulmonary hypoplasia continues to be the major cause of death in newborn infants with diaphragmatic hernia. Recent investigations have suggested that postnatally induced pulmonary injury can result from excessive positive or negative intrathoracic pressure and contribute to the respiratory deterioration. Therefore, the method of thoracic drainage on the side of the diaphragmatic hernia is critical in controlling and maintaining normal intrathoracic pressure in both intrathoracic spaces. No chest tube or an ipsilateral chest tube connected to water seal, can result in either excessive negative or positive intrathoracic pressure and, therefore, both methods should be avoided. Recently, we employed a "balanced" intrathoracic drainage system which maintains the ipsilateral intrathoracic pressure within the normal physiologic range of +2 to -8 cm H2O regardless of the degree of pulmonary hypoplasia, presence of an ipsilateral pulmonary air leak, straining by the infant, or mechanical ventilation. This system is simple, requires no suction apparatus, and is easily assembled with equipment readily available within the hospital. This technique has been utilized in 18 newborn infants with diaphragmatic hernia and pulmonary hypoplasia. There have been no complications which specifically could be related to the balanced drainage system.

Pregnancy and breast cancer.

Breast cancer in association with pregnancy and lactation is rare, but presents a therapeutic problem of considerable magnitude. The outlook for such patients is less favorable than that of nonpregnant, nonlactating women, probably because the stage of the disease is more advanced when it is discovered. The most significant factor in the poorer prognosis is physician delay in diagnosis and therapy. When mastectomy is carried out early in pregnancy, the operation can be as effective as in nonpregnant women of the same age groups. It is emphasized that when pregnancy and breast cancer are found concurrently, prompt therapy for the cancer should be undertaken. Interruption of pregnancy in nondisseminated breast cancer is of little value. If pregnancy is near term when the diagnosis of disseminated breast cancer is made, the desire of the husband and wife for a child should be considered. A modest delay in therapy to allow for delivery probably has no deleterious effect. Castration should be withheld and used only for the patient with metastatic disease. There may be a place for prophylactic castration in the treatment of disseminated disease, but its role is yet to be clearly defined. Subsequent pregnancies in a patient with axillary spread at the time of mastectomy are contraindicated, because of the high rate of treatment failure and decreased rate of survival. In patients desiring future pregnancies following mastectomy, a period of observation of at least 2 years seems wise. At the end of that period, if clinical evaluation, laboratory values, roentgenographic studies, and isotopic bone scanning are negative for disseminated disease, subsequent pregnancies seem safe. Prompt evaluation of any breast mass found during pregnancy and lactation should be carried out by needle or operative biopsies under local anesthesia. Although the prognosis of the pregnant or lactating woman with breast cancer is generally favorable, numerous long-term survivals are encountered in those women who undergo prompt mastectomy early in pregnancy. The former pessimistic outlook for such patients seems unjustified. With modern methods of diagnosis and treatment, therapy can be effective and successful.