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A term newborn developed signs and symptoms of an upper-intestinal obstruction. A palpable transverse upper-abdominal mass was identified. An ultrasound examination demonstrated a cystic mass with massive dilatation of the biliary tree. At laparotomy, dozens of segmental intestinal atresias (IA) from the jejunum to the rectum were identified. The cystic mass was the duodenum, obstructed distally from the atresias and proximally from an antral web. The biliary dilatation was due to normal drainage into a closed-loop duodenal obstruction, and was relieved by division of the web. The multiple atresias were explored, but establishment of intestinal continuity was impossible. The entity of multiple segmental IAs in association with cystic biliary dilation has been previously described, but there are no recorded survivors; 35 patients have been reported in the world literature. Our patient was the longest survivor; she finally died after 2 years of gastrostomy-tube drainage and total parenteral nutrition while awaiting intestinal transplantation. Only 5 patients other than ours had documented immune-system abnormalities. We review the findings and management of this rare syndrome.

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Necrotizing enterocolitis (NEC) primarily affects premature newborns. Regional and national decreases in the mean birthweight and gestational age of neonatal intensive care unit (NICU) admissions prompted a review of NEC in VLBW (very low birth weight, defined as < 1,000 g) infants in our institution over a 25-year period. There were 266 patients treated for NEC during the study interval. We compared 71 VLBW with 195 non-VLBW infants and found that VLBW infants were: fed later (6.4 days v 4.1 days, P = .009), developed NEC later (20.8 days v 13.1 days, P = .002), had significantly lower 1- and 5-minute Apgar scores, were more likely to require surgery (51% v 34%, P = .016), more often had panintestinal (defined as > 75% of intestinal length) involvement (10% v 4%, P = .043), and had poorer survival (56% v 72%, P = .013). Overall survival after NEC has improved over the study interval, both in our series and in other reports. However, the increasing number of VLBW infants who have NEC represent a subgroup who appear to be generally more ill, develop NEC later, require surgery with greater frequency than their non-VLBW counterparts, and are less likely to survive.

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Necrotizing enterocolitis (NEC) is a syndrome of diverse etiologies with a significant mortality rate affecting mostly prematurely born stressed infants. Now recognized as a discrete entity, it had been poorly defined because other conditions seem to represent the same entity. A number of risk factors have been identified that appear to "trigger" NEC, though these have been questioned because they have been present just as frequently in premature and older infants who did not develop NEC as in those that did. Recently, maternal cocaine use has been added to the suspected risk factors. A steady improvement in the survival of babies with NEC has been due largely to a high index of suspicion of the disease and early, aggressive medical management.

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Anterior cricoid resection is an effective procedure to relieve subglottic stenosis. This is well documented in adults, although reports of the procedure in growing airways are limited. Over an 11-year period, seven pediatric patients underwent anterior cricoid resection for recalcitrant subglottic stenosis. In four patients, the stricture was secondary to prolonged intubation, one developed subglottic stenosis following a high placement of tracheostomy for epiglottitis and another had congenital subglottic stenosis. One child had subglottic stenosis combined with laryngotracheoesophageal cleft and more distally located tracheoesophageal fistula. All patients had failed to respond to previous treatment: dilatations (3 to 20), steroid injection (3 patients), and Evan's tracheoplasty (2 patients). All patients had an excellent result from anterior cricoid resection. The median age of children undergoing anterior cricoid resection was 3 years. There was no mortality. Tracheostomy decannulation was accomplished within 12 weeks following operation in all patients. It was necessary to remove a tracheal granuloma in one patient. Anterior cricoid wedge resection leaving the posterior portion of the cricoid in place is done to avoid recurrent nerve injury. It is a relatively simple and effective procedure. There has been minimal morbidity and no mortality. Follow-up from 1 to 11 years shows no recurrence of stenosis. There has been normal laryngeal and airway growth.

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Using a posterior repair and rectal suspension procedure for those patients who need surgical treatment of rectal prolapse, we have treated 46 patients over a period of 17 years at Children's Mercy Hospital in Kansas City, MO. One patient with caudal dysgenesis died of multiple congenital anomalies following two unsuccessful attempts at posterior repair and suspension. Four patients developed a recurrence afterwards, which was found to be due to sigmoid intussusception and, presumably, had played a major part in their original prolapse. Two of these required resection, one from the transanal approach and one from the transabdominal approach. One resolved spontaneously and another is as yet unresolved. Three patients had minor mucosal prolapse that was transient and two patients had extrusion of silk sutures but continued to have a very satisfactory result. Overall, 42 patients had satisfactory resolution of their rectal prolapse. Three of the four patients who had unsatisfactory results had associated anomalies that contributed to their poor outcome.

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There are conflicting views on the pathogenesis of the intestinal malfunction seen in infants with gastroschisis. It has been variously ascribed to abnormalities of ganglion cells and smooth muscle elements, intestinal ischemia, and the "peel" which invests the serosa of the intestine. Review of the clinical and experimental literature showed only limited information on the histology of the eviscerated human intestine. In order to add to this data base, and to further investigate the pathogenesis of the intestinal malfunction from a histologic standpoint, we reviewed surgical and autopsy material from our experience with 105 neonates with gastroschisis. Ten specimens were satisfactory for evaluation from a standpoint of tissue integrity. The specific mural components of mucosa, submucosa, muscularis, and ganglion cells were examined and found to be either normal, or to show nonspecific abnormalities that varied from case to case, and were related mostly to intestinal infarction due to compromise of the gut at the site of the gastroschisis defect. In six patients, this progressed to atresia formation. The most consistent abnormalities were found in the serosal layer with its peel. Using special stains, the peel was found to be composed largely of fibrin and collagen. Based on this study, we feel that edema and ischemic changes, though often present, are much less prominent than the peel, as the leading histologic abnormality of the intestine of gastroschisis. Squamous epithelial cells were seen in the peel in four cases, suggesting that the peel had been "appliqued" onto the serosa of the herniated fetal gut.

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One hundred consecutive patients with esophageal atresia or tracheoesophageal fistula, or both, were treated at The Children's Mercy Hospital during the past 14 years. Each patient was evaluated and a plan for therapy was formulated depending on the type and urgency of concomitant disease. Healthy patients were treated by primary repair. Those with respiratory disease were treated before repair by gastrostomy, upper pouch suction, and antibiotics. In the small premature infant and the patient with major associated anomalies, repair of the esophageal atresia and tracheoesophageal fistula was postponed by gastrostomy, upper pouch suction, and parenteral nutrition while the concomitant disease was treated. Fifteen patients with life-threatening associated anomalies required operation before esophageal repair and 13 of them survived. During the initial hospitalization two deaths related to esophageal atresia occurred, whereas three prerepair and two late (2 1/2 and 3 months) postrepair deaths related to associated anomalies occurred. Thus, 93 of the 100 patients left the hospital alive. The increased survival is due not only to improved care of esophageal atresia and tracheoesophageal fistula, but also to a more aggressive approach to the treatment of the serious associated anomalies.

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Two patients were observed who had transient quadriceps paresis following local inguinal block for postoperative pain control following inguinal herniorrhaphy.

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Ureteropelvic junction (UPJ) obstruction is a common cause of hydronephrosis in infants. Newborns with severe obstruction often have marked improvement following correction; therefore early diagnosis and operation is important. From 1973 to 1983, 21 patients were operated on for UPJ obstruction diagnosed under 6 weeks of age. Six patients (29%) had antenatal ultrasonographic diagnosis. The remaining patients were diagnosed by IVP or radionuclide scan for palpable renal enlargment or for associated anomalies. Seventeen had unilateral and four had bilateral obstruction. Twenty-three pyeloplasties, one primary nephrectomy, and one cutaneous pyelostomy with subsequent nephrectomy were done. All pyeloplasties were dismembered, with tailoring of the renal pelvis. Postoperative renal function was followed with radionuclide scan or IVP. Postoperative complications included a single urinary tract infection in three patients and two bowel obstructions. One early postoperative death occurred in an infant with bilateral obstruction who developed congestive heart failure secondary to severe uncontrollable hypertension. There were two other unrelated late deaths. Documented functional improvement with minimal complications follow unilateral or simultaneous bilateral pyeloplasty in newborns with UPJ obstruction.

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The technique of anterior fundoplication for gastroesophageal reflux originally described by Alan Thal has been used in our institution in 605 patients. The description of the technique is the purpose of this paper. A brief description of the results in these 605 patients are also presented.

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Neonatal sepsis due to group B beta-hemolytic Streptococcus (GBS) is reported to occur in about 1 out of 330 live births. Right-sided Bochdalek hernia (RBH) occurs in about 1 of 20,000 live births. The combination of group B streptococcal sepsis and delayed appearance of a right Bochdalek hernia is an infrequently reported phenomenon--18 patients have been previously reported in the English literature. We add four patients from our own experience to these previous reports. Since approximately 10% to 15% of the newborn population are exposed to group B Streptococcus we suspect that the inadequate diaphragmatic motion on the side of the Bochdalek hernia predisposes the child to development of septicemia and/or pneumonitis. Once the etiology has been established and appropriate antibiotic therapy instituted, progressive improvement in the patient's course should be seen. This is in contrast to a very significant mortality rate in many of the patients having early onset GBS. Any child, therefore, surviving early onset GBS only to deteriorate again, should be suspected of having an associated right Bochdalek hernia, and diagnostic steps should be taken to evaluate the integrity of the right diaphragm.

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Ecchymosis of the scrotum and lower abdominal wall occurred in four newborn boys. All were anemic. Three had coagulation abnormalities and evidence of sepsis. In two, group B streptococcal septicemia was documented. Intraperitoneal hemorrhage from a ruptured subcapsular hematoma of the liver was the source of blood in the scrotum in three, and most probably in the fourth as well. Two infants died in spite of antibiotics, vigorous blood replacement, including exchange transfusion, and desperation laparotomies for continued intraperitoneal hemorrhage. Newborns with scrotal ecchymosis should be examined for intraperitoneal hemorrhage, ruptured subcapsular hematoma of the liver being the most probable source. Their coagulation status should also be evaluated, and sepsis should be suspected, especially in those with a demonstrated coagulopathy. Group B Streptococcus is a likely primary etiologic agent in these critically ill neonates. Nonoperative treatment, as given the two survivors in this experience, is preferred.

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Two critically ill neonates with severe renovascular hypertension as a complication of aortic monitoring catheter are presented. They did not respond to intensive medical therapy for hypertension. In spite of their precarious general condition, nephrectomy was undertaken with complete relief of symptoms.

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The anterior fundoplication described by Thal has been used in treating gastroesophageal reflux surgically in 362 children at The Children's Mercy Hospital and at St. Luke's Hospital in Kansas City, Missouri, because medical therapy had failed or was inappropriate. Long-term results have been evaluated in regard to relief of reflux and relief of symptoms attributed to reflux. Of the 335 patients followed from 1 to 8 years, 90% had a satisfactory initial result. Five percent required reoperation for a recurrence of reflux due to failure of the fundoplication or development of a hiatus hernia. All recurrences developed with 5 months of the initial operation. Fifteen of 335 patients (4.5%) had persistent symptoms despite correction of the gastroesophageal reflux; in these patients, attributing the symptoms to reflux was incorrect. There were no deaths in this series of patients as a result of operation. The success rate of the Thal fundoplication in children compares favorably with that of the Nissen Fundoplication.

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