Frank G. DeLuca

Malignant potential in intestinal juvenile polyposis syndromes

AbstractBackground: Unlike familial polyposis coli, where the premalignant nature of adenomatous polyps is well established, the cancer risk in juvenile polyposis has generally been considered not increased. Methods: This study reviews all cases of juvenile polyposis reported in the English language to date to assess the occurrence and prognosis of carcinoma in the gastrointestinal tract. Results: A total of 218 patients met the inclusion criteria. Mean age at diagnosis was 18.5 years (range: 9 months to 67 years). No gender preference was identified. The most common presenting symptom was chronic anemia, followed by acute gastrointestinal bleeding, rectal prolapse of polyp, protein-losing enteropathy, and intussusception. A family history of juvenile polyposis could be established in ∼50% of patients, and associated congenital malformations were detected in 15%. Ninety-nine patients underwent 138 gastrointestinal operations: 121 colorectal, 12 gastric, and 5 small intestinal procedures. The development of a gastrointestinal carcinoma was reported in 36 cases (17%). Mean age at diagnosis of carcinoma was 35.5 years (range: 4–60 years). Most malignancies were located in the distal colon and rectum, with only one case of gastric and one case of duodenal carcinoma. Tumor stage at diagnosis was usually advanced, with poor survival figures. Conclusions: This study shows that juvenile polyposis syndromes carry a more significant risk of carcinoma than generally appreciated. Therefore, more intense endoscopic surveillance may be warranted, and definitive surgical options should often be considered in these syndromes.

Incidence of contralateral inguinal hernia: A prospective analysis

BACKGROUND/PURPOSE Contralateral groin exploration in children with unilateral inguinal hernia is still controversial, particularly in infants. The authors have attempted to determine the age- and gender-stratified incidence of contralateral hernia and the necessity of routine bilateral procedures. METHODS This is a prospective study of 656 patients during a 34-month period at a single institution. Patients with unilateral hernia underwent an ipsilateral procedure only, regardless of age, gestational age, or gender. Follow-up was 6 to 40 months (mean, 25.5 months). Chi-square analysis was used for intergroup comparison (P < .05 significant). RESULTS Of 656 children, 108 (16.5%) presented with synchronous bilateral hernias. Bilateral inguinal hernia was significantly more common in premature infants (28.0%) and young children (33.8% if <6 months, 27.4% if <2 years). Of the remaining 548, a metachronous contralateral hernia developed in 48 (8.8%) at a median interval of 6 months (range, 4 days to 7 years). This incidence was 13 of 105 (12.4%) in infants less than 6 months of age, 20 of 189 (10.6%) in children less than 2 years of age, 8 of 54 (14.8%) in premature infants, 6 of 81 (7.4%) in girls, and 8 of 29 (27.6%) in children with an incarcerated hernia. In the latter group, P < .05, chi2 analysis. CONCLUSION Routine contralateral inguinal exploration, without clinical evidence of a hernia, may be advisable in children with incarceration and possibly in premature infants. The low incidence of contralateral hernias in all other patients, regardless of gender or age, does not justify routine contralateral exploration.

Gardner's syndrome; a revisit to a previously described family.

The complete pedigree of the family with Gardners syndrome, first reported by Weiner and Cooper, is presented and the natural history of this autosomal dominant disorder is reviewed. Two new cases were found among members of the third generation. Fifteen years after total colectomy, the only affected survivor from the second generation developed a metastasizing adenocarcinoma of the third portion of the duodenum. In addition to features of the classic triad, the dental abnormalities found in all affected survivors in this family appear to be a consistent manifestation of the syndrome. No structural or numerical chromosome abnormalities were found in karyotypes prepared from peripheral white blood cells, a mandibular osteoma or testicular cells. All affected individuals should be advised of the high risk of producing affected offspring. Careful evaluation of all family members at risk will allow identification of affected individuals long before intestinal symptoms develop and should permit appropriate surgical therapy before malignant degeneration of the colon can occur. Until the true incidence of malignancy in the retained rectum is established, subtotal colectomy with careful lifelong follow-up would appear to be the most acceptable therapy for Gardners syndrome. Even total colectomy may not be curative, since the small bowel mucosa carries a small but definite premalignant potential.

Neonatal septum transversum diaphragmatic defects

Over 10 years, 8 infants required surgery for central diaphragmatic herniation. Contrast peritoneography and technetium-99m-sulfur colloid radionuclide scanning were the most definitive diagnostic aids. Associated anomalies included variations of the pentalogy of Cantrell. A midline gastroduodenal loop was found in two infants. Six infants are alive and well 6 months to 3 years postoperatively. An abdominal approach is preferred if there is an intestinal hernia, associated gastrointestinal anomalies, or if a bilateral defect is present.

External compression as initial management of giant omphaloceles

The authors describe a noninvasive technique for the management of giant omphaloceles. Two patients with giant omphaloceles were managed with external compression. Dry sterile dressings were used, buttressed by an Ace bandage in the first case and by a handcrafted Velcro abdominal binder in the second. The binder was tightened every 2 or 3 days. Renal, cardiovascular, respiratory, and gastrointestinal parameters were measured regularly to determine whether the binder was too tight. The first patient had only occasional emesis, and the defect was repaired after 40 days of compression. The second patient experienced intermittent hypertension, occasional emesis, and mild oxygen desaturation, which resolved when the binder was loosened slightly. The fascia muscle and skin were closed after 30 days of external compression. Both patients are currently living at home and doing well. This form of external compression is an effective, inexpensive, and low-risk method for the gradual reduction of giant omphaloceles, and should be considered for patients born with this problem.

Child abuse as a cause of traumatic chylothorax

Chylothorax is an uncommon condition that may be associated with significant morbidity and mortality. The authors report a case of traumatic chylothorax attributed to child abuse and describe our management with tube thoracostomy and nutritional support with medium-chain triglycerides. Child abuse should be suspected in any case of chylothorax when no other etiology is evident and particularly when other signs of abuse are present.

Use of an elemental diet in the nutritional management of catabolic disease in infants

Abstract Elemental diets have proved useful in the nourishment of infants and children with gastrointestinal tract dysfunction. Newborn and premature infants, because of their immature gastrointestinal tracts and kidneys, are very sensitive to the elemental diet concentration and flow rates. They do not tolerate concentrations of elemental diets of greater than 10 per cent w v . This, however, provides 0.4 cal/ml and 2.40 mg protein nitrogen/100 ml. In children older than ten months, solutions up to 25 per cent w v are well tolerated if the diet is started slowly as a continuous 15 per cent w v feeding and then gradually advanced in speed and concentration over several days to a week or more to allow for gastrointestinal adaptation. Careful monitoring of weight, hematocrit, electrolytes, and urine sugar are important to guard against the development of hyperosmolar dehydration. Vitamin K must be given, since diets do not contain it, and patients will otherwise become hypoprothrombinemic. Elemental diets would appear to be useful in pediatric patients with massive small bowel resection, exocrine pancreatic insufficiency, fistulas of the gastrointestinal tract, malabsorption, food allergies, inflammatory bowel disease, and in conditions of markedly increased energy requirements such as burns, sepsis, and surgery.

Urethral Polyps in Male Children

One of the rare causes of intrinsic obstruction of the urethra in male children is the urethral polyp. Only two examples of this condition, one of which recurred six years after the original excision, have been recorded in our pediatric hospital. This rarity is consistent with the few cases reported in the current literature. In general, review articles on obstructive lesions of the lower urinary tract in children fail to mention polyps of the posterior urethra (11, 12). Campbell (1), Kjellberg (2), Rudhe (3), Flanagan (4), McCann (5), and Scott (6) report and discuss a single case, each. True et al. (7) discuss 2 cases, and Stephens (8) reports 5 in male children. The same abnormality has been described in an adult with secondary hydronephrosis by Barrie and Simms (9). None of these authors reported recurrence of a urethral polyp. Case Reports Case I: A 5-year-old boy had a single episode of hematuria one year prior to admission. One month before admission, symptoms of dysuria, pyuria, and frequency, as ...

Positive 99mTc-Pertechnetate scan in a child with intestinal arteriovenous malformation

A teenager with massive rectal bleeding had a positive 99mTc-pertechnetate abdominal scan. At laparotomy, an arteriovenous malformation of the jejunum was found. There was no evidence of a Meckels diverticulum. The persistent problem of the positive technetium scan warrants increased utilization of preoperative visceral angiography.

A home program of long-term total parenteral nutrition in children†

Three children--ages 4 months, 5 months, and 14 years--have been on a program of total parenteral nutrition at home for ten, 23 and 44 months respectively, as of January, 1978. Using a specially designed silicone rubber catheter, placed in the right atrium, total nutritional needs of these children were delivered nightly by family members; the children carried out normal activity during the day with the catheter line maintained by a heparin lock. Normal skeletal development and weight gain have been achieved while allowing these children normal social and psychlogic development outside the hospital. During the course of the therapy the patients had multiple metabolic abnormalities which were successfully treated by replacement therapy. The duration of catheter patency ranged from three to 22 months. Catheter sepsis or mechanical failure occasionally required catheter removal and replacement.