Inhaled nitric oxide in congenital hypoplasia of the lungs due to diaphragmatic hernia or oligohydramnios.

OBJECTIVE: We determined whether inhaled nitric oxide (NO) could improve systemic oxygenation in human neonates with hypoplastic lungs. METHODS: A multicenter nonrandomized investigation was performed to study the efficacy of short-term NO inhalation. Inhaled NO was administered at 80 ppm to nine neonates without evidence of structural cardiac disease by echocardiography. Lung hypoplasia was due to congenital diaphragmatic hernia (CDH) in eight patients and to oligohydramnios in one patient. A total of 15 trials of NO inhalation were performed in these nine patients. Eight trials in seven patients were performed before extracorporeal membrane oxygenation ((ECMO); one patient had two trials) and seven trials were performed in five patients after decannulation from ECMO (two patients had two trials each). RESULTS: NO inhalation before ECMO did not change postductal PaO2 (42 +/- 3 mmHg vs 42 +/- 4 mmHg), oxygen saturation (SpO2; 89% vs 88%) or oxygenation index (31 +/- 4 cm H2O/torr vs 31 +/- 4 cm H2O/torr) for the group. All patients required ECMO support, which lasted from 5 to 17 days (mean 9). After decannulation from ECMO, NO inhalation increased postductal PaO2 from a median of 56 mm Hg (range 41 to 94) to a median of 113 mm Hg (range 77 to 326), P < .05. It decreased the oxygenation index from a median of 23 cm H2O/torr (range 11 to 7) to a median of 11 cm H2O/torr (range 4 to 21), P < .05. It increased SpO2 from 91% to 96% (P < .05) and pH from 7.48 +/- .03 to 7.50 +/- .03. CONCLUSION: In our patients with hypoplastic lungs, inhaled NO was effective only after ECMO. This could be due to maturational changes such as activating the endogenous surfactant system. Inhaled NO may be effective in neonates with hypoplastic lungs who have recurrent episodes of pulmonary hypertension after ECMO, even if they were previously unresponsive.

Urinary tract anomalies associated with congenital diaphragmatic defects.

Anomalies of the urinary tract occur in some 13-27% of infants with congenital posterolateral diaphragmatic defect (CDD) and are often severe (renal agenesis, dysplasia, hypoplasia, or hydronephrosis). To test the hypothesis that urinary and diaphragmatic anomalies share elements of pathogenesis, we reviewed 60 autopsy cases of CDD studied at our institution. Sixteen patients (27%) manifested anomalies of the urinary tract: 12 had markedly altered kidneys, 8 of which were unilateral and ipsilateral to the diaphragmatic defect. Among 27 patients free of gross urinary tract anomalies, kidney weights formed a skewed distribution, with most values above published norms for body weight; by analysis of covariance, kidney weight (as a function of body weight) was significantly greater in CDD than in a control population of infants free of chronic illnesses and congenital anomalies who died suddenly and unexpectedly. Differences in glomerular number and diameter could not be identified between the latter groups. In 71% of patients with isolated left CDD, the left kidney was heavier than the right, a reversal of the usual condition in infancy. These findings demonstrate that both marked and subtle changes of the urinary tract in CDD are generally ipsilateral to the diaphragmatic defect and suggest that the pathogenetic mechanisms responsible for urinary and diaphragmatic anomalies may overlap topographically. Aberrant morphogenesis within a developmental field or fields is one explanation for this.

Abdominal abscess. A surgical strategy.

To reassess the role of laparotomy and extraserosal drainage in the treatment of patients with abdominal abscess, we analyzed the course of 79 patients who underwent 97 operations to treat 120 abdominal abscesses during a five-year period. In 66 clinical episodes the abscess was drained by the most direct approach. Sepsis resolved with a single operation In 80% of these patients, five patients (8%) required a second operation for drainage for an abscess, and eight patients (12%) died. In 31 clinical episodes, the abscess was drained by a laparotomy. Sepsis resolved with a single operation in 61% of these patients, seven patients (21%) had a second abscess, six patients (19%) required a second operation to drain a metachronous abscess, and six patients (19%) died. When the location or number of abscesses was diagnosed incorrectly, the success rate of therapy fell substantially. Since most abdominal abscesses can now be accurately diagnosed preoperatively, most abscesses should be drained by a direct approach. Exploratory laparotomy is indicated when preoperative localization is unsuccessful, when sepsis has not resolved after other methods of drainage, or when the patient has a concomitant abdominal condition that must be treated surgically.

Results of a pilot trial comparing prolonged intravenous antibiotics with sequential intravenous/oral antibiotics for children with perforated appendicitis.

HYPOTHESIS: For children with perforated appendicitis, the use of a prolonged course of intravenous (i.v.) antibiotics is equivalent to a short course of i.v. antibiotics followed by sequential conversion to oral (PO) antibiotics. DESIGN: Prospective, randomized, clinical trial. SETTING: Multicenter study in tertiary children's hospitals. PATIENTS: Children (aged 5-18 years) with perforated appendicitis found at laparotomy. INTERVENTION: Children were randomized after appendectomy either to a 10-day course of a combination of i.v. ampicillin, gentamicin sulfate, and clindamycin (n = 10); or to a short course of a combination of i.v. ampicillin, gentamicin, and clindamycin, followed by conversion to a combination of p.o. amoxicillin and clavulanate potassium plus metronidazole (n = 16). MAIN OUTCOME MEASURES: The primary outcome measure was clinical success, which was rated as complete, partial, or failure. Secondary outcome measures included return of oral intake, duration of fever, return of normal white blood cell count, and patient charges. Treatment equivalence was determined using confidence interval analysis. RESULTS: We found treatment equivalence between the i.v. and i.v./p.o. groups, with 6 (60%) complete and 4 (40%) partial successes for the 10 patients in the i.v. group and 15 (94%) complete and 1 (6%) partial successes for the 16 patients in the i.v./p.o. group (P< or =.05). There was no difference in return of oral intake, duration of fever, or return of normal white blood cell count between the groups. Conversion to oral therapy results in savings of approximately $1500 per case. CONCLUSION: There is treatment equivalence between prolonged i.v. therapy and i.v. therapy followed by conversion to oral antibiotic therapy in children with perforated appendicitis.

Iodine toxicity in a patient treated by continuous povidone-iodine mediastinal irrigation.

Continuous povidone-iodine irrigation is frequently used to treat mediastinitis after median sternotomy and has been considered safe and effective. We describe a 34-month-old patient with mediastinitis after median sternotomy who was treated with continuous povidone-iodine irrigation and who absorbed toxic quantities of iodine (total serum iodine, 9,375 micrograms/dl; normal range, 4.5 to 9.0 micrograms/dl). An unexplained metabolic acidosis developed, along with changes in mental status, and the patient died. This experience and a thorough review of the literature lead us to believe that continuous povidone-iodine irrigation of the mediastinum is contraindicated.

Patterns of enamel maturation.

Investigations of the maturation pattern of rat incisor enamel by quantitative electron microscopy and electron-probe microanalysis indicate that mineralization of rat enamel can be regarded, as in humans, as a regular and progressive process pattern of enamel matrix formation. The species variations that have been proposed in the pattern of enamel mineralization can be related to differences in both the rate of formation of the enamel matrix and in the total thickness of the enamel produced. Neither the microradiographic appearance of developing enamel, nor the solubility and staining characteristics of the organic matrix accurately reflects the mineral concentration gradients established during the mineralization process as indicated from electron microprobe analysis.

Kaolin-induced congenital hydrocephalus in utero in fetal lambs and rhesus monkeys.

A model of congenital hydrocephalus in utero in fetal lambs and rhesus monkeys has been produced by the intracisternal injection of kaolin. Initial attempts to produce hydrocephalus using silicone oil were unrewarding. Hydrocephalus had developed by 2 weeks post-injection and could be followed by ultrasonography. The pathological findings were similar to those reported using kaolin in other species. Ventriculoamniotic shunting, when successful, was capable of partially reversing the deleterious effects of hydrocephalus. The major drawback of the present model is that hydrocephalus is produced during the second rather than the first trimester of pregnancy. However, kaolin produces mainly an obstructive hydrocephalus without other associated brain or systemic anomalies.

Individualized management of colonic atresia.

Atresia of the colon represents the least common cause of neonatal intestinal obstruction, comprising less than 10 percent of gastrointestinal atresias. Eleven patients underwent surgical repair of colonic atresia during a 5 year period. Five of the patients were premature. Two had associated gastroschisis and other congenital anomalies were present in three patients. Hypaque enema was utilized to diagnose intestinal atresia and laparotomy was then undertaken. Primary repair could be performed in four patients, one with type I and three with type 3 atresias. These four patients did well and were discharged within 13 days after operation. Three deaths occurred in patients with associated anomalies, yielding a mortality rate of 28 percent. Operative therapy of colonic atresia should be individualized, based on the condition of the patient at presentation and the presence of associated birth defects.

Pediatric hernias and hydroceles.

Hernias and hydroceles are common conditions of infancy and childhood, and inguinal hernia repair is one of the most frequently performed pediatric surgical operations. As a result of improved neonatal intensive care, more and more premature babies are being delivered, and consequently the incidence of neonatal inguinal hernia is increasing. The most important aspect of the management of neonatal inguinal hernias relate to its risk on incarceration, and emphasis is placed on this point. This article covers the embryology, incidence, clinical presentation, and treatment of groin hernias and hydroceles, as well as dealing with abdominal wall hernias other than umbilical hernias. This article places special emphasis on when a patient with a hernia or hydrocele should be referred to a pediatric surgeon.

Fluid therapy for the pediatric surgical patient.

The fluid management of the pediatric surgical patient is a crucial aspect of surgical care. This article reviews the fundamental physiology of fluid replacement in children and highlights how standard formulas for fluid therapy can be modified to account for the rapidly changing physiology of the pediatric surgical patient. Novel approaches to fluid treatment of the surgical patient with oral rehydration formulas are discussed. Finally, guidelines for specific management of common pediatric surgical diseases are presented.

Pediatric umbilical problems.

After birth, the normal umbilicus is a relatively simple structure. During the development of the embryo, however, this region is highly complex. Vestigial of the umbilical cord can be responsible for umbilical inflammation and drainage. This article reviews the embryology of the umbilicus and discusses a number of clinical problems seen in this area. The authors' aim is to aid the primary care pediatrician in evaluating, treating, and appropriately referring umbilical problems encountered in office practice.

Antenatal diagnosis of pediatric surgical anomalies. Counseling the family.

Improvements in screening and diagnostic techniques now mean that hundreds of congenital anomalies can be antenatally diagnosed. It is, however, impossible and inappropriate to submit all pregnant women to a barrage of investigations. Screening is necessary before specific invasive investigations are initiated. These include history, physical examination, MS-AFP screening, estriol and hCG screening, and a Level II ultrasonography scan. Once at-risk pregnancies have been identified, a multidisciplinary team approach is commenced and further studies including Level II ultrasonography, amniocentesis, chorionic villus sampling, or cordocentesis can be performed so that an accurate diagnosis is available. Counseling of the parents throughout is essential so that appropriate decisions regarding this and further pregnancies can be made.

Choledochal cysts: a ten year experience.

Choledochal cyst (CC) is a rare disorder that usually presents in childhood. Prognosis depends on early diagnosis, complete excision of the cyst, and reconstruction by hepaticojejunostomy. This report details our 10-year experience and emphasizes innovations in our management. Sixteen patients presented with CC at a mean age of 3 years. (Range, newborn to 21 years, with a M:F ratio 1:4). Two groups could be identified on the basis of age at presentation. Group I (N = 7), presented in the neonatal period, three with obstructive jaundice and four without symptoms. In Group II (N = 9), all patients presented with ascending cholangitis at a mean age of 6 years. Thirteen patients had a type 1 CC, one patient had a type 3 CC, and two had type 4 CC. The patients with type 1 and type 4 CC underwent primary cyst excision with Roux-en-Y hepaticojejunostomy, whereas the patient with type 3 CC underwent cyst excision with sphincteroplasty of the ampulla. There was only one complication of postop cholangitis that cleared with antibiotic therapy. All patients have remained free from symptoms in the follow-up period (6 months-10 years). Our four most recent cases were diagnosed in utero by prenatal ultrasonography. This led to appropriate antenatal counseling and prospectively planned neonatal surgery. These infants were asymptomatic, with no clinical signs at birth, and antenatal ultrasonography prevented diagnostic delay. Excision of the choledochal cyst and primary hepatico-enteric anastomosis is confirmed as the therapy of choice. Antenatal sonography is a sensitive method in the diagnosis of CC and offers the opportunity for early diagnosis and planned surgery before the onset of complications.

The approach to common abdominal diagnosis in infants and children.

This article focuses on salient points in the evaluation of abdominal pain in infants and children. Specifically, the authors address appendicitis and abdominal pain associated with either vomiting, constipation, or gastrointestinal bleeding. A discussion of common abdominal masses, urologic, and gynecologic problems, and considerations in the evaluation of immunologically suppressed or neurologically impaired children, and children with recurrent abdominal pain is also presented. The authors establish logical, focused approaches to the initial evaluation and management of abdominal pain and suggest criteria for timely surgical referral.

The approach to common abdominal diagnoses in infants and children. Part II.

Part I (August 1998 issue, Pediatric Clinics), discussed appendicitis and common abdominal diagnoses in infants and in children associated with vomiting, as well as special considerations in the evaluation of immunologically suppressed and neurologically impaired pediatric patients. In this article, the authors continue to discuss the evaluation of constipation, gastrointestinal bleeding, common abdominal masses, and recurrent abdominal pain.

Care of the surgical intensive care nursery graduate. The primary care pediatrician's perspective.

Care of the intensive care nursery graduate may be quite challenging. It is important that primary care pediatricians become familiar with the complications unique to surgical patients so that they may properly prepare and educate parents and provide appropriate long-term follow-up for these often complex patients. Maintenance of a close relationship with the pediatric surgeon with an open line of communication regarding the approach to various surgical problems facilitates the effective integration of the intensive care nursery graduate into the primary care pediatrician's practice and provides the foundation for a successful clinical outcome.

Cardiopulmonary consequences of congenital diaphragmatic hernia.

The cardiopulmonary consequences of CDH can be devastating, and despite intensive research the pathophysiologic makeup of this condition is not fully understood. It seems likely that its overall prognosis is related to the size of the pulmonary vascular bed and its capacity to accept the cardiac output at birth. The ability of the lungs to perform this task is impaired by a surfactant deficiency and by pulmonary vessels that respond excessively to normal stimuli of vasoconstriction. Understanding the mechanisms through which these pathophysiological abnormalities are occurring should provide us with therapeutic insights into how we may recruit all the available alveoli in these lungs, prevent ventilation-induced lung injury, optimize pulmonary blood flow, and maximize cardiac output without causing right or left heart compromise. Failure to achieve all or any of these goals can feed back and exacerbate the underlying pathophysiological condition.

Congenital diaphragmatic hernia. A historical review.

Congenital diaphragmatic hernia (CDH) has been described, studied, and treated for over 400 years. A historical perspective is given. Great strides have been made in the care of patients with CDH; however, the mortality rate remains 30% to 60%. Consideration of historical references, observations, and achievements in the study of CDH is important for the continued advancement and improvement in our understanding of and future success in treating infants and children with CDH.

The fetal heart in diaphragmatic hernia.

Cardiac evaluation is an important part of the investigation of a fetus with a diaphragmatic hernia. The heart is displaced by the physical presence of the abdominal contents in the chest, and the flow dynamics within the heart are altered. This can produce secondary diminution of chamber growth, particularly of the left heart. Structural heart malformation is also a frequently associated finding. Evaluation of cardiovascular flow profiles may give us further understanding of the pathophysiology of this lesion and may help to predict those fetuses that might benefit from intrauterine therapy.

Postnatal management of congenital diaphragmatic hernia.

The emphasis in postnatal management has shifted from the neonatal surgical emergency approach to strategies designed to deal with pulmonary hypoplasia and the pulmonary vascular abnormalities. There has been extensive experience with alternative ventilation strategies such as ECMO and high frequency ventilation, without there being convincing evidence that these have had a major impact on mortality. Strategies that emphasize the importance of minimizing ongoing lung injury, such as pressure limited (permissive hypercapnia) ventilation and the use of surfactant replacement therapy, are beginning to show some encouraging results.