Delayed repair of congenital diaphragmatic hernia with early high-frequency oscillatory ventilation during preoperative stabilization.

PURPOSE: The authors reviewed their experience in the management of CDH after the introduction of early high-frequency oscillatory ventilation (HFOV) during the preoperative stabilization period and delayed CDH repair. METHODS: This is a retrospective analysis of 24 consecutive infants with CDH treated at University of California, Irvine Medical Center (UCIMC) during a 36-month period from January 1993 to December 1996. RESULTS: Two patients were excluded from the study: one fetus with a prenatal diagnosis was referred for fetal surgery; one infant received CDH repair at another institution 2 weeks before transfer to UCIMC. Eight (36%) infants were inborn, and nine (41%) had a prenatal diagnosis of CDH. Median gestational age was 40 weeks (range, 29 to 42 weeks). Median birth weight was 3,019 g (range, 1,205 to 4,337 g). The defect was left sided in 18 infants (86%). Twenty-one infants were intubated within 5 hours of life, 15 had an AaDO2 greater than 610, 11 had an oxygenation index greater than 40, and 11 had a pH of less than 7.2. The median ratio of pulmonary artery pressure to systemic blood pressure was 0.93 (range, 0.51 to 1.15) in 12 infants. Eighteen infants were placed on HFOV within a median of 1 hour of life. Nitric oxide was given to six infants and surfactant to eight. Four infants were referred for extracorporeal membrane oxygenation (ECMO). Repair of CDH was performed on infants at a median age of 33.5 hours (range, 5.5 to 322). Six (30%) received a prosthetic patch. Overall 18 of 22 infants survived (81%); three survivors received ECMO. Two infants of the survivor group had congenital heart anomalies: one ventricular septal defect (VSD) and one double-outlet right ventricle with a VSD. Of the four nonsurvivors, one had lethal cardiac anomalies and bilateral CDH, two had severe bilateral pulmonary hypoplasia (one received ECMO), and one infant was a 29-week premature baby who did not qualify for ECMO. CONCLUSION: We report a survival rate of 81% (18 of 22) with the management of CDH by delayed surgical repair, early postnatal HFOV, and selective referral for ECMO.

The importance of family functioning to caregiver adaptation in mothers of child cancer patients: testing a social ecological model.

This study was designed to investigate associations between family relationships, personal-psychological caregiver adaptation (ie, depressive symptomatology, sense of burden), and ability to function within the health care system (perceived doctor-parent relationship) in mothers of child cancer patients. Data were collected through a survey of a sample of 29 mothers whose children were diagnosed with some form of childhood cancer, mainly leukemia. Family functioning was positively related to both caregiver adaptation and doctor-parent relationship. Mothers who reported decreased maternal depression and burden were significantly more likely to report respectively improved spousal relationships and improved spousal communication. Mothers reporting more positive doctor-parent relationships also described themselves as having improved relationships with spouse and child, and improved spousal communication. Maternal well-being and doctor-parent relationship were not directly related. By suggesting that both intrapsychic and instrumental maternal adaptation are influenced by positive relationships in the family, this study provides support for the social ecological model of stress. The possibility that family factors exert a direct influence on both caregiver intrapsychic well-being and instrumental skills argues for the importance of their being carefully considered in any models evaluating caregiver adaptation.

Cytomegalovirus enteritis in a premature infant.

BACKGROUND/PURPOSE: Up to 2.5% of newborn infants are cytomegalovirus (CMV) positive at birth. Five percent will be symptomatic at birth, including cytomegalic inclusion disease. Symptoms such as hearing loss and mental retardation will ultimately develop in 15%. METHODS: The authors describe a case of CMV enteritis in a 2.2-kg newborn that presented as necrotizing enterocolitis (NEC) and subsequently developed a colonic stricture. RESULTS: There are four reports of neonatal CMV enteritis in the nonEnglish-language literature. Cytomegalovirus enteritis has become prevalent among the immunosuppressed pediatric and adult patient population. CONCLUSIONS: We propose the addition of CMV to the list of pathogens responsible for NEC. A review of neonatal CMV infection is provided.

Focal intestinal perforation in the extremely-low-birth-weight infant.

The purposes of this report were to (1) document the clinical and laboratory features of 11 extremely-low-birth-weight (ELBW) infants with focal intestinal perforation and (2) investigate the clinical events possibly associated with these perforations by examining matched pairs of infants with and without focal intestinal perforation. During the study period 173 infants with birth weights between 600 and 1000 gm were admitted to the neonatal intensive care nursery. Eleven of these ELBW infants had focal intestinal perforations and formed the study group. These infants were matched with 11 ELBW infants who did not have intestinal perforations or signs of inflammatory bowel disease. The matched pairs were similar in all respects except for a significantly higher percent increase in blood urea nitrogen level after treatment with indomethacin (Wilcoxon signed-rank test, p < 0.02) in infants with intestinal perforation. At laparotomy the perforations were noted to be focal, often multiple, and on the antimesenteric border of the distal ileum. None of the infants showed clinical, radiographic, or intraoperative findings that were consistent with classifications for necrotizing enterocolitis (NEC). The incidence of focal intestinal perforation in ELBW infants was 6% versus 2% for typical NEC. In addition, four of the 11 infants with intestinal perforation had positive cultures for either Staphylococcus epidermidis or Candida albicans, whereas none of the infants without perforation had positive cultures during the study period (Fisher's exact test, p < 0.09). We conclude that the clinical presentation and the characteristic intestinal lesions in this group of ELBW infants are distinct from those in typical cases of NEC.(ABSTRACT TRUNCATED AT 250 WORDS)

Use of magnetic resonance imaging in planning the separation of omphalopagus conjoined twins.

Magnetic resonance imaging (MRI) was used for the first time in the preoperative planning for separation of conjoined twins. In these omphalopagus infants, MRI showed normal biliary and cardiovascular structures and demonstrated, in detail, a relatively avascular plane through the liver bridge, which enabled safe separation with minimal blood loss at 3 1/2 months of age. A single MRI study supplied information superior to that obtained with multiple previously available imaging studies. MRI should be an essential part of the preoperative workup of all types of conjoined twins.

The surgical management of hyperinsulinism in infancy due to nesidioblastosis.

Severe neonatal hypoglycemia due to nesidioblastosis demands prompt diagnosis and treatment to prevent mental retardation. Early central venous catheter placement is essential for a constant glucose infusion. At surgery, near-total (95%) pancreatectomy is done, starting at the tail and preserving the spleen. Bipolar electrocoagulation is very useful for the tiny vessels. The uncinate process is removed leaving a small amount of pancreas adjacent to the preserved common bile duct. Three patients, diagnosed shortly after birth, had surgery at 34 days, 2 years, and 17 days of life. Two patients developed staphylococcal infections, one of whom exhibited the "scalded baby" syndrome and required reoperation for evisceration. Insulin was required for one to seven days in two and for three months in one. Diazoxide was needed for 18 months in the initial patient, who did not have uncinate resection. All patients are healthy and off medication with a postoperative follow-up period of 11, 12, and 65 months.

Endoscopy versus double-contrast barium enema in the evaluation of patients with symptoms suggestive of colorectal carcinoma.

Two hundred seventy-six patients with positive findings on a stool guaiac test, hematochezia, a change in bowel habits, or a family history of colorectal carcinoma were referred to the colorectal clinic for further investigation. There were 105 men and 171 women. All patients underwent examination with the 65 cm flexible sigmoidoscope. Patients with no abnormalities on endoscopy underwent a double-contrast barium enema. If the barium enema revealed a polyp or other suspicious pathologic process, the patient was referred for colonoscopy. In 258 patients, findings of barium enema and flexible sigmoidoscopy were in agreement. The findings in 178 of these examinations were completely negative, and in the remaining 80 cases flexible endoscopy and barium enema revealed diverticulosis. In an additional 18 patients, negative findings on flexible sigmoidoscopy were followed by positive findings on barium enema. Thirteen of these 18 patients (72 percent) had negative findings on colonoscopies and therefore had false-positive findings on barium enema. In the remaining five patients (28 percent), an unsuspected colonic carcinoma or premalignant polyp was discovered on barium enema and documented by colonoscopy. Also, in four of these five patients, colonoscopy found additional polyps that were not seen on barium enema.

Alternative operative procedure for total colonic aganglionosis.

The authors have utilized a technique recently described by Boley as the corrective procedure in two patients with complex long segment aganglionosis. The one-stage operation combines a right colon onlay patch for enhanced absorptive and reservoir purposes with an ileoendorectal pull-through. Both patients had only 3 to 4 stools per day by the end of the first postoperative month. The obligatory period of intestinal adaptation needed to achieve an acceptable stooling pattern is significantly reduced in comparison with results obtained with other commonly used procedures.

The role of colonoscopy and flexible sigmoidoscopy in screening for colorectal carcinoma.

Six hundred thirty-two patients were referred to the Colorectal Clinic from February 1983 to February 1986 for screening with the Pentax 65 cm flexible sigmoidoscope. Forty-nine of these patients (8 percent) had adenomatous polyps. There were 27 males and 22 females. The mean distance examined by the 65 cm flexible sigmoidoscope was 55 cm. Five patients were excluded from analysis, leaving 44 patients who underwent colonoscopy to the cecum. At the time of colonoscopy, 15 of the 44 patients (34 percent) had one or more adenomatous polyps beyond reach of the 65 cm flexible sigmoidoscope. The remaining 29 patients who underwent colonoscopy had no polyps beyond reach of the 65 cm flexible sigmoidoscope. Thirty adenomatous polyps, one invasive carcinoma of the ascending colon, and one hyperplastic polyp were found in these 15 patients. In summary, 34 percent of patients found to have adenomatous polyps within reach of the 65 cm flexible sigmoidoscope harbored one or more adenomatous polyps in the proximal colon at the time of colonoscopy. A positive 65 cm flexible sigmoidoscope examination requires colonoscopy to identify and remove proximal premalignant lesions, thereby aborting the polyp-cancer sequence.

Interval breast cancer: a more aggressive subset of breast neoplasias.

It has been suggested that interval breast cancer (breast cancer found within 12 months of a negative screening examination) exhibits a more virulent behavior than those found by the screening clinic. To test this hypothesis, 120 new cases of breast cancer were retrospectively reviewed from our Breast Screening Center. These patients were divided into three groups--interval cancer cases, lesions found at initial screening, and lesions found at subsequent screening. Statistically significant differences seen in our interval cancer cases included a higher percentage of positive axillary nodes, higher overall mortality rate, and lower 6-year survival rate as calculated by left table analysis. The remaining data, although not statistically significant, demonstrate a parallel trend toward a more malignant behavior. It is concluded that interval breast cancers are more aggressive forms of breast neoplasias.

Hemodynamic effects of prolonged infusion of prostaglandin E1 (PGE1) after hemorrhagic shock.

Although one-hour infusions of prostaglandin E1 to animals suffering from hemorrhagic shock have proved beneficial, there is question as to whether the improved hemodynamics can be sustained over a longer infusion time. Following reinfusion of shed blood, an infusion of PGE1 at a dose of 1 microgram/kg/minute showed a significant elevation in cardiac output and left ventricular stroke work and a decrease in systemic vascular resistance when compared to animals receiving only an equal volume of normal saline. There was no diminution in the hemodynamic activity of the drug over the entire infusion period. These data indicate that the drug might be appropriate for hemodynamic support in the clinical situation, where infusion times of greater than one hour are often necessary.

Small bowel reversal procedure for treatment of the "short gut" baby.

An increasing number of infants have undergone massive intestinal resection for various reasons: uncontrollable diarrhea, malabsorption, and failure to thrive makes these babies a difficult management problem and threatens their survival and later "quality of life." Five short gut babies have had a 3-cm small bowel reversal operation as part of the total salvage effort. It is recognized that very skilled care by dedicated personnel and parents, utilizing i.v. nutrition and special feeding techniques, is probably the most significant factor in achieving success with these babies. It is believed small bowel reversal helps in keeping these infants alive while their intestinal tract is making the adjustments necessary for long-term survival.

Thyroid surgery in children and teenagers.

From 1961 to 1976 62 patients under age 20 underwent thyroidectomy for various thyroid disorders. Twenty-four thyroid lobectomies and eight subtotal or near-total thyroidectomies were performed for benign nodular goiter. Eight near-total thyroidectomies and two thyroid lobectomies were performed for carcinoma. Two patients also had a radical neck dissection. Twenty patients with hyperthyroidism underwent near-total thyroidectomy. Postoperative complications occurred in six patients-all with hyperthyroidism. Operative mortality was zero. Two indications for thyroidectomy in our series were nodular goiter (to rule out carcinoma), and hyperthyroidism (that was not well-controlled medically or where surgery was chosen as primary therapy). In patients with nodular goiters that required surgery, a minimal complication rate occurred. By contrast, surgery for hyperthyroidism was associated with a high postoperative complication rate, six of 20 patients or 30%, which must be anticipated by the surgeon.