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A retrospective review of 1,220 cystic fibrosis (CF) patients between 1965 and 1989 identified 60 patients who underwent appendectomy. Ten had appendectomy prior to referral and 16 had an incidental appendectomy (primarily meconium ileus). Among the remaining 34 patients, acute appendicitis was present in 19 (1.8% incidence). "Classic appendicitis"--acute abdominal pain shifting to the right lower quadrant (RLQ), focal RLQ tenderness, and elevated white blood cell (WBC) count--was present in 15. Four additional inflamed appendixes were removed in patients following incorrect preoperative diagnosis. Thirteen of these 19 were perforated. Complications included wound infection (2), pelvic abscess (1), ileal obstruction requiring ileostomy (1), and pelvic hematoma (1). There were no deaths. One patient with acute RLQ pain and tenderness had a normal appendix. Seven patients (mean, 20 years) had chronic, intermittent, focal RLQ pain and tenderness not originating periumbilically with a normal WBC count and temperature. At exploration, these appendixes were enlarged and tensely distended with inspissated mucus. Microscopic examination showed no inflammation. Appendectomy resulted in resolution of symptoms without complications. Four additional patients with intermittent RLQ pain and tenderness and a history of recurrent intussusception presented with ileocolic intussusception. Persistent postreduction symptoms in three and failure of reduction in the fourth necessitated celiotomy at which time tensely distended appendixes were removed. Appendectomy led to resolution of symptoms. Three additional enlarged noninflammed appendixes were incidentally removed in asymptomatic patients undergoing unrelated intraabdominal procedures. Appendiceal disease in CF patients represents a spectrum ranging from simple mucous distention to acute appendicitis with perforation. CF patients with pain secondary to a noninflamed distended appendix represent a distinct syndrome cured by appendectomy.(ABSTRACT TRUNCATED AT 250 WORDS)

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This prospective study compared the efficacy of sulbactam/ampicillin and clindamycin/gentamicin in the treatment of children with bacterial peritonitis. Of the 29 children enrolled, 17 were evaluable; eight received sulbactam/ampicillin/gentamicin and nine clindamycin/gentamicin. Sixteen patients were previously healthy children with appendicitis. An average of 3.6 bacterial species were recovered from the peritoneal fluid of each patient. E coli and B fragilis were the most common aerobic and anaerobic isolates, recovered from 15 and ten patients, respectively. Pseudomonas aeruginosa was recovered from seven of 17 children; the three children with P aeruginosa infections randomized to the sulbactam/ampicillin group received gentamicin in addition to the investigational agents throughout the treatment course. Although the study groups were small, there was no difference in age, sex, number of pathogens per patient, duration of hospitalization, toxicity, or treatment failures between the two treatment groups or between children infected with P aeruginosa and controls. As a result of the high prevalence of P aeruginosa in the peritoneal exudate of otherwise healthy children with appendicitis, initial antimicrobial therapy in this patient population should include agents effective against this organism.

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A 15-year review of children's hospital patients with cystic fibrosis (CF) who underwent surgery yielded 578 cases in 210 patients (mean 2.7 per patient). The median age was 16 years (range newborn to 43 years). Four hundred procedures were done under general anesthesia and 176 under local. There was one anesthetic complication, respiratory depression in a patient whose MediPort (Cormed, Inc, Medina, NY) was inserted using local anesthesia and sedation. The most frequent procedure was nasal polypectomy, with 165 procedures in 50 patients. The second most common procedures were vascular access procedures: 75 central lines and 29 MediPorts were implanted in 57 patients, complicated by two pneumothoraces. Thoracic procedures included 32 bronchoscopies, 8 lobectomies, 2 pneumonectomies, and 30 pleural strippings. There were three reoperations for bleeding in the pulmonary resection patients. Thirteen newborns underwent a total of 26 procedures for meconium ileus and its complications, with two deaths secondary to respiratory failure and sepsis. These, and one death postlobectomy were the only operative deaths in the entire series of 578 cases (0.5% mortality rate). There were four slings for rectal prolapse; two required removal secondary to infection. Eight patients underwent central splenorenal shunts for portal hypertension, 15 underwent cholecystectomy, 5 underwent Nissen fundoplication, 16 underwent inguinal herniorrhaphy, 2 underwent umbilical herniorrhaphy, 3 underwent orchidopexies, and 4 underwent miscellaneous pediatric surgical procedures. Eleven patients underwent appendectomy for appendicitis; four were ruptured at the time of diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

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During a 52-month span, 14,324 ultrasonographic examinations were performed on 9,453 pregnant patients. One-hundred and fifty-one anatomical malformations were found in 122 fetuses (1.29%). Our analysis of patients referred to the perinatal center for ultrasonography indicates that the number of high risk patients has increased, and a parallel increase of neonatal surgical anomalies has resulted. An analysis of fetuses concluded that anomalies of the: gastrointestinal tract had improved care, deaths occurred due to associated anomalies or severe prematurity; genitourinary system received earlier diagnosis and treatment; central nervous system/musculoskeletal system/hydrops--no difference in management, treatment or outcome was noted; teratoma/cystic hygroma--did not effect treatment; cardiovascular system--inutero medical treatment by digitalization of the mother was possible. Paradoxically, an increase in the mortality of diaphragmatic hernia patients was noted and concluded to be secondary to the extremely early detection of this anomaly.

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Stomal prolapse is a common complication of colostomies and, if severe, may affect the timing of the definitive procedure. A simple technique for postreduction bowel fixation without stomal revision is presented. The reduced bowel is attached to the parietal peritoneum using a "U" type suture a few centimeters from the stoma. Two short parallel segments of latex tubing prevent the thread from cutting through. Suture and bolsters are removed when adhesions are established. In one of the four children, a percutaneous cecostomy was employed to connect a prolapse of the entire proximal colon.

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A critically ill 20-month-old white male with severe bronchopulmonary dysplasia, cor pulmonale, and intolerance to enteral feeds, required a left percutaneous subclavian Broviac catheter. Echocardiogram showed a right atrial thrombus, resolution of which was demonstrated after infusion of streptokinase through the Broviac catheter for 16 hours. The catheter remained in place, functioning well, for a further 4 months. The patient suffered no bleeding complications, and was spared the prohibitive risk of right atrial thrombectomy. This use of streptokinase has not previously been reported.

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In an effort to eliminate the common problems associated with gastrostomy tubes in children, we developed a silicone rubber gastrostomy "button" designed to replace the standard long latex tube. Easily inserted and self-retaining, the device remains practically flush with the skin. The intragastric portion is similar to a dePezzer catheter, and a one-way valve prevents reflux of gastric contents. Prototypes were evaluated in five children for a time period of 3 to 18 months. In two additional patients with esophageal stricture, the device was used as a temporary plug to maintain gastric stoma patency. Parents and nurses are pleased with the button's low profile, appearance, and ease to use. It is neither painful nor irritating, and there is no fear of dislodgement.

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In a 4-year span, 12 patients with hepatic trauma were treated at our institution. After initial resuscitation, three underwent emergency celiotomy because of the severity of their injuries. This communication analyses the other nine. All of these had significant liver injuries and were managed nonoperatively. Hepatic injury was diagnosed and the severity defined by ultrasonography, radionuclide scintigraphy, or computed tomography (CT). These stable patients were initially admitted to the intensive care unit (ICU) and upon transfer to the ward, maintained on restricted activities. Seven of the nine children received blood transfusions. Although liver enzymes were initially elevated in each case, they returned to normal in 1 week. In-hospital and post-discharge imaging documented healing of the hepatic injuries in each case. Eight children are well at 3 months to 4 years follow-up. Only one of the nine nonoperatively managed patients died and this was secondary to severe head injuries. To date, there are no complications. Our nine children are added to the 23 nonoperatively treated pediatric hepatic injuries found in the literature.

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A newborn with rectal atresia underwent a sigmoid colostomy followed by fluoroscopic placement of a string across the membrane. Progressive dilatations were done until normalization of the rectal canal occurred at 4 months. At 6 months, the colostomy was closed. Although moderate sigmoid dilatation persisted initially, the boy is now developing well and has a normal stooling pattern 3 years following the last procedure. The technique demonstrates the feasibility of high rectal-membrane management without excision.

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The outcome of 139 fetuses with anatomical abnormalities diagnosed by ultrasonography (out of 15,180 examinations) at our institution during the last 6 years was reviewed. Of these, 56 were diagnosed before 21 weeks gestational age and 83 thereafter. Fifty-four had anomalies of the central nervous system. Of these 27 were diagnosed early. Twenty-six had anomalies of the genitourinary tract, 11 diagnosed early. Four had anomalies of the musculoskeletal system diagnosed early in 3. Thirty-seven had miscellaneous anomalies and early diagnosis was made in 6. Only the early pregnancies were terminated, 38 in number. The fetal diagnosis was clearly confirmed in 33. Of particular interest were the 18 fetuses with anomalies of the gastrointestinal tract and abdominal wall. There were nine early diagnoses. The pregnancy was interrupted in seven. In all the diagnosis was confirmed. Five of these had associated anomalies. Among the 9 fetuses whose anomalies were diagnosed late, 3 had associated anomalies and died shortly after birth before corrective surgery. In all, two fetuses with abdominal wall defects had no detectable associated anomalies and were potentially correctable but the pregnancies were terminated. With earlier, accurate imaging diagnosis possible, special attention has to be directed toward multidisciplined counseling (including a pediatric surgeon) for ultimately satisfactory fetal-neonatal salvage. Otherwise the spectrum of neonatal surgical pathology could be seriously altered.

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A newborn female had ileal atresia without mesenteric continuity associated with a small, shortened colon but without "malrotation." After correction by an ileocolostomy the infant remained obstructed, requiring an ileostomy at 24 days; subsequently, at 13 months, a Martin-Duhamel operation was done. since associated anomalies are uncommon in each of these conditions, since no cause and effect relationship has been developed, and since no prior survival has been clearly documented, this report is important. The recommendation of distal bowel biopsy is stressed to establish an early diagnosis and, thereby, correct treatment.

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Although anastomotic leaks following retropleural esophagoesophagostomies for esophageal atresia are usually followed by spontaneous closure, disruptions may be life-threatening because of inability to control oral secretions. This communication describes the use of a distally placed transanastomotic tube to control severe leakage in three patients.

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Two female siblings died within three months after presenting with paravertebral tumors in the first year of life. The pathology of the two tumors was identical and characteristic of a malignant rhabdoid tumor. There were no identifiable tumor patterns within the kindred which have been associated with any hereditary cancer or precancer syndromes. Fibroblasts were cultured from skin biopsies obtained from the second patient and both parents. Assays of growth kinetics associated with cellular transformation revealed that fibroblasts from the affected sibling can be distinguished from those of the parents and age-matched controls by increased in vitro occurrence of tetraploidy. Such evidence suggests that increased in vitro tetraploidy occurring spontaneously in cultured fibroblasts is an expression of a cancer-prone gene. Increased in vitro tetraploidy has previously been demonstrated in some kindreds with heritable colon cancer syndromes, and may extend our understanding of the genetic etiology of some childhood cancers.

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In three patients with neuroblastoma and high circulating levels of dopamine and dopa, we interfered pharmacologically with catecholamine biosynthesis either at the tyrosine hydroxylase or dopa decarboxylase step in an attempt to 1) improve the efficacy of antitumor therapy and 2) avoid the potential arrythmogenic interaction between elevated circulating catecholamines and an halogenated hydrocarbon anesthetic during surgery. Biochemical evidence indicated that inhibition of catecholamine biosynthesis had occurred but there was no associated significant change in clinical status or response to other therapy.

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A 7-yr-old sustained a gunshot wound of the upper abdomen lacerating the portal vein and liver requiring immediate repair. Postoperative angiograms demonstrated two false aneurysms of the aorta between the renal arteries and the superior mesenteric artery. On the thirteenth postoperative day the aneurysms were resected and a Dacron graft interposed between the level of the renal arteries and the celiac axis. The superior mesenteric artery was reattached utilizing a short segment Dacron graft. The boy made a good recovery and is normal after a 3-yr follow-up.

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With the greatly expanded indications for central venous lines in children, and especially with the introduction of the Broviac silastic catheter, a new, safe, and fast method of catheter insertion has been developed. The method combines the advantages of direct major vessel approach and the use of a Broviac catheter. Originally Broviac et al. (1973) suggested insertion of the catheter by either a subclavian venipuncture or by a cephalic vein catheterization. The latter technique is commonly used, even though technical difficulties are not infrequent. Our method is based on a modified Seldinger catheter introduction employing an 8 French Peel Away Sheath Set for the adult Broviac catheter and a 5 French set for the pediatric catheter. Forty-six catheters were placed in 43 consecutive patients ranging in age from 2 mo to 21 yr. Perioperative complications were minimal and late catheter related complications few. An increase in the number of oncologic patients was observed.

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Six infants with necrotizing enterocolitis were discharged after periods of prolonged hospitalization (two to nine months) with intact ileostomies. Their initial hospitalization was complicated by feeding difficulties, chronic diarrhea, sepsis, rickets, and developmental delay. All were rehospitalized within three months, with severe acidosis and dehydration after a presumed viral-type illness. Each had large-volume ileostomy output, which was rich in electrolytes and bicarbonate. A prolonged recovery phase (two to eight months) again was punctuated with episodes of diarrhea, problems in starting oral feeding, and sepsis. After reanastomosis of the remaining bowel, no infant has had a similar life-threatening episode. It is speculated that the infants' recurrent "salt-and-water-losing states" are secondary to either an anatomic or functional loss of the colon. This problem appears to be a poorly recognized sequela of bowel surgery and necrotizing enterocolitis, and early reanastomosis of discontinuous bowel should be of benefit.

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Rectal prolapse occurred in 112 (18.5%) of 605 cystic fibrosis patients. In 48 patients prolapse preceded diagnosis of cystic fibrosis, but physicians (pediatricians, pediatric and general surgeons, and proctologists) rarely appreciated its importance as a symptom of this disease. Prolapses frequently cease with institution of pancreatic enzyme replacement therapy following diagnosis of cystic fibrosis. However, even when the disease remains undiagnosed, the symptom is often transient and frequently resolves at 3-5 yr of age. Prolapse occurring initially after cystic fibrosis is diagnosed rarely responds to manipulation of diet or enzyme doses. Many patients develop a method of reduction which involves voluntary abdominal, perineal, and gluteal muscles and does not require manual pressure on the prolapsed segment. Most patients do not need specific treatment for the prolapse. Surgery is rarely necessary. A sweat test should be obtained on any child who has had even a single episode of rectal prolapse.

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In transverse or crossed testicular ectopia, the affected gonad lies in the same canal as the normally descended testis. During a 5-yr span, three children with this form of ectopia were seen. All presented with a symptomatic right inguinal hernia and an empty scrotum on the left side. The ages at operation were 1, 3, and 5 mo. Only the first patient was reoperated. In this child, the diagnosis of transverse testicular ectopia was made during the herniorrhaphy and the ectopic, but otherwise normal, gonad returned to the abdominal cavity. A subsequent left orchidopexy through a celiotomy was done. In the last two patients, the correct diagnosis was made preoperatively. Both gonads were of equal size and normal, occupying the same hemiscrotum. A herniorrhaphy with fixation of the ectopic gonad to the opposite hemiscrotum was done in both. All three children are otherwise normal. Cases collected from the literature are discussed. The condition should be suspected if a unilateral hernia is associated with a contralateral, nonpalpable testis and may not be as rare as formerly thought.

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