Imperforate anus: an unusual presentation of a common disease.

Imperforate anus is a common problem facing the pediatric surgeon. Most cases of imperforate anus are discovered at birth during the initial physical examination and are corrected early. The pediatric population also accounts for 80% of all cases of ingested foreign bodies. Most of these pass through spontaneously and the treatment is observation. In this article, we present the case of a seven-month-old child in whom failure to pass a swallowed coin led to the diagnosis of imperforate anus. This case illustrates an uncommon presentation of a common disorder and emphasizes the importance of vigilance and careful physical examination in the newborn.

Neonatal Wilms tumor.

Neonatal renal tumors are very rare. We have identified 15 cases of Wilm's tumors diagnosed in the first 30 days of life. The most common presentation was an abdominal mass on routine newborn examination, and three tumors were noted on an antenatal ultrasound. All of the infants underwent primary excision of the tumor. Five infants were not given postoperative chemotherapy after surgical excision. One of these children had a relapse and later died of progressive disease. The remaining children are alive at a median follow-up of 31 months. Eight of the tumors had evidence of nephrogenic rests. More frequent follow-up physical and imaging examinations are indicated in children not given adjuvant chemotherapy or whose tumors show nephrogenic rests.

Does the current health care environment contribute to increased morbidity and mortality of acute appendicitis in children?

To determine whether the current "gatekeeper" controls on health care lead to an increase in treatment delay and morbidity of acute appendicitis in children, we reviewed the experience with this disease at a large children's hospital over a 10-year period. One hundred seven consecutive children 18 years and younger operated on for acute appendicitis from July 1, 1988 to June 30, 1990 were compared with 119 children with the same diagnosis from July 1, 1978 to June 30, 1980. Age, sex, race, antecedent illnesses, initial physician contact and diagnosis, time to referral and operation, pathology, morbidity, and length of stay were reviewed. The two groups were comparable in terms of age, sex, race, antecedent illnesses, and negative appendectomy rate. More patients in the recent group were initially seen in an emergency room or urgent care setting than in the previous group (62.2% v 48.5%, P = .07). The accuracy of the initial diagnosis was significantly lower in the more recent group (P = .05). No change existed between the groups in the time to a physician; however, a significant (P = .04) difference existed in the time to surgeon (41.2 hours in the earlier group v 56.4 hours in the recent group). No significant difference existed between the groups in time from surgeon to operation. Although not statistically significant, the morbidity rate was increased in the recent group (13.3% v 6.5%, P = .17).(ABSTRACT TRUNCATED AT 250 WORDS)

Pneumatosis cystoides intestinalis in children beyond the first year of life: manifestations and management.

Beyond infancy, pneumatosis cystoides intestinalis (PCI) is rare. Data concerning pathogenesis and treatment are limited. Our experience with 12 children was examined to define predisposing factors, presentation, treatment, and outcome. Nine children were immunosuppressed, thus identifying an important etiologic subgroup. Presentation was variable but included abdominal pain, distention, diarrhea and hematochezia. Clostridium difficile was found in 3 patients and cytomegalovirus in 1. Radiographs showed free air in 3. Nine were treated with antibiotics and bowel rest, 1 with bowel rest alone, 1 with oral metronidazole, and 1 with observation. PCI resolved in 7 of 9 treated with antibiotics, although 1 child with leukemia had severe hematochezia secondary to colonic ulceration and required hemicolectomy. No other patient required laparotomy. The free air resolved in 2 of 3. There were 2 deaths, both from sepsis. One had free air on admission but no perforation was found at autopsy. Treatment recommendations remain unclear; however, C difficile and cytomegalovirus are important pathogens that should be identified and treated promptly. In symptomatic patients, bowel rest and antibiotics seem beneficial. Operative intervention should be reserved for patients with peritoneal signs, progressive deterioration, obstruction, or persistent, severe bleeding. Free air alone is not an indication for operative management in children with PCI.

Dihydrocapsaicin (DC) protects against serum hyperlipidemia in guinea pigs fed a cholesterol-enriched diet.

Juvenile guinea pigs were maintained either on a standard diet (cholesterol-free) or a 0.5% cholesterol-enriched vitamin C deficient diet for six weeks. Half of the animals of each dietary group were treated with dihydrocapsaicin (DC) at the rate of 8 mg per animal per day. DC administration significantly altered food consumption, body weight, and dry weight of feces of animals maintained on the standard diet. In addition, the mean serum triglyceride concentration of these animals was significantly decreased with DC treatment. Food consumption, body weight and (wet) fecal weight of animals maintained on the 0.5% cholesterol-enriched diet were significantly lower than the corresponding values in control animals even though the liver was much larger in proportion to the total body weight. The mean serum cholesterol concentration of the animals fed a cholesterol-enriched diet was significantly elevated. However, in animals also receiving DC, the cholesterol and triglyceride levels were comparable to controls fed a regular diet. The gross and microscopic fatty infiltration of the liver observed in the cholesterol-fed animal were minimized in those animals fed cholesterol and DC.

Myo-osseous intercostal pedicle flaps for tracheal reconstruction in puppies.

We evaluated a myo-osseous intercostal pedicle flap for distal tracheal reconstruction. Mongrel puppies, 6 to 10 weeks old, underwent tracheal repair, 12 for primary defects and seven for stenotic lesions created in the distal trachea. A composite flap was constructed from the anterior portion of the fourth rib with the overlying pleura and periosteum and a posteriorly based intercostal muscle/neurovascular pedicle. The animals did well following tracheal reconstruction without evidence of airway obstruction. Bronchoscopy documented normal tracheal diameter and the repair site could not be discerned from the surrounding mucosa. Histologically, there was complete respiratory epithelial ingrowth with no inflammatory changes. The rib graft had active hematopoietic marrow. This work suggests that the myo-osseous intercostal pedicle flap provides a surface for normal epithelial ingrowth of tracheal mucosa without stimulating granulation tissue, interposes a stent capable of growth, therefore minimizing anastomotic stricture, and is an effective alternative in the management of distal tracheal stenosis.

Gastroesophageal reflux and the premature infant.

Gastroesophageal reflux (GER) is a well-recognized problem in infants and children. Only scant mention of the premature infant with GER can be found in the literature. Of 760 preterm infants admitted to the NICU between 1980 and 1984, 22 had documented GER. These infants all underwent medical management including upright positioning, small frequent feeds, and often, nasojejunal feedings. Seventeen babies did not respond to medical management and underwent surgical therapy to control the reflux. Of the 17 babies requiring fundoplication, 15 had been initially intubated for treatment of respiratory distress syndrome. Eight of these 15 were extubated in less than 25 days and were improving until they exhibited sudden episodes of deteriorating pulmonary status requiring reintubation. The other seven intubated patients developed striking bronchopulmonary dysplasia (BPD) in the first month and required prolonged ventilatory support. Pulmonary deterioration, failure to grow, and refusal to eat became the herald of GER in these infants. Fundoplication dramatically improved the pulmonary status in all but one infant. Three late deaths can be attributed to cor pulmonale and pulmonary failure. BPD was striking predisposing factor for severe GER in these premature infants. In the total premature population without BDP only 8 of 684 (1.2%) had GER with five responding to medical management and three others undergoing fundoplication for apnea-bradycardia spells. Fourteen of the 76 infants with BPD (18.4%) had significant GER and all required surgical management for control of symptoms. Premature infants who develop deteriorating pulmonary function, poor growth, and/or refusal to eat should be evaluated for GER.

Wilms' tumor in the neonate: a report from the National Wilms' Tumor Study.

Renal neoplasms in the neonate are quite uncommon. Twenty-seven of the 3,340 patients (0.8%) registered on the National Wilms' Tumor Studies from 1969 through April 1984, were 30 days old or less. Of these 27 patients, 18 had mesoblastic nephroma, 1 had a malignant rhabdoid tumor of the kidney, and 4 others had nonneoplastic lesions. The remaining four infants were reviewed in detail. All had favorable histology Wilms' tumors; none had distant metastasis at diagnosis. Treatment ranged from surgery alone to excision plus three-drug therapy for 15 months. All fared well. The patient with Stage I rhabdoid tumor died at eight weeks of age in spite of aggressive four-drug therapy. This review supports the view that Wilms' tumor in the neonate is extremely rare.

Acalculous cholecystitis in the neonate.

Pediatric acalculous cholecystitis is a well-described though uncommon affliction. However, during the neonatal period nonlithogenic biliary disease is extremely unusual and only isolated reports of this problem exist. Nine previously reported cases are reviewed, and an additional case of a 6-day-old female neonate with a right-upper-quadrant mass is presented in detail. Inflammatory gallbladder disease in the neonate is often associated with concurrent systemic illness. An abdominal mass is palpable in almost all cases. Diagnosis may be confirmed in these patients by radionuclide scanning and ultrasonography. The treatment is cholecystectomy.

Patterns of pediatric gastroesophageal reflux.

Gastroesophageal reflux (GER) in the pediatric patient is a frequently recognized problem. Unlike the adult, in whom symptoms relating to esophagitis predominate, the infant and child may present with a variety of respiratory problems, vomiting and/or growth failure. GER is often seen in association with other conditions and must be considered in the evaluation of any pediatric patient with chronic recurring respiratory problems, vomiting or failure to thrive (FTT). Thirty-eight pediatric patients have been surgically managed at West Virginia University from 1977-1983 for GER. The patients fall into several different patterns of presentation and associated problems. Nine premature infants all with bronchopulmonary dysplasia (BPD) have undergone fundoplication for FTT, worsening BPD, and pneumonia. Seven infants and two older children had GER associated with previous esophageal atresia repairs. Esophagitis, vomiting and growth failure were the predominant complaints in this group, though all nine patients had recurring respiratory symptoms as well. Syndromes involving mental retardation and neurologic dysfunction affected another group of five patients, all of whom presented with the complications of long-term esophagitis. The remaining 15 children were otherwise healthy infants who had predominantly respiratory symptoms due to GER. The benefits of fundoplication in these severely affected infants and children far outweigh the relatively few complications. In the carefully selected patient, surgical management of GER is dramatically successful.

Changing management in familial polyposis. Role of ileoanal endorectal pull-through.

Total colectomy with ileoproctostomy was performed in 32 members of a family spanning three generations and ranging in age from 10 to 54 years. In seven of these patients (22 percent) carcinoma developed in the retained rectum over a median follow-up period of 14 years. This high incidence of rectal carcinoma has demanded reevaluation of treatment recommendations in patients with polyposis coli. Ten patients aged 7 to 30 years have undergone total abdominal colectomy with ileoanal endorectal pull-through since 1980. All were one stage procedures without reservoir construction. Within 3 months the patients all had good control with 5 to 10 semiformed stools daily and had resumed normal activities. Follow-up date shows adequate dilatation of the distal ileum and no evidence of polyps. Total colectomy and ileoanal endorectal pull-through are effective treatment for familial polyposis in patients of all ages. It should be considered the primary procedure in new patients and an excellent method of converting those patients who have ileoproctostomy to a safer situation.

The occurrence of carcinoma of the rectum following ileoproctostomy for familial polyposis.

Ileoproctostomy was performed in 32 patients (13 Female and 19 male), with polyposis coli ranging in age from 10 to 54 years. Seven patients (22%) developed cancer of the retained rectum with a median follow-up of 14 years. Two (20%) of ten patients, followed for 10 to 15 years, and three (50%) of six patients, followed for 15 to 20 years, developed rectal cancer. Rectal cancer developed in two of 14 patients who had their ileoproctostomy at 14 cm and in five of 18 patients who had their ileoproctostomy at a higher level, with a median followup of 7 and 11 years, respectively. Rectal cancer developed in two of 15 teenage patients undergoing ileoproctostomy and in nine of 17 patients aged 20 to 54 years. The present average ages of the two groups were 25 and 41 years, and the average age at which rectal cancer appeared was 40 years. Three of the patients who developed rectal cancer had numerous polypectomies over the years, and there was a tendency to develop tubulovillous and villous adenomas with a variable degree of atypia leading to carcinoma. One patient also showed a return to high levels of coprostanol and secondary fecal bile acids. Proctocolectomy, if acceptable, may be the treatment of choice; ileoproctostomy may mean that the patient eventually will undergo a proctectomy. The ileoanal endorectal pull-through procedure has a great deal to offer to these patients, and further study is necessary to evaluate this procedure.

Catheter embolization: preparation of patient with pheochromocytoma.

This is a case report of a patient treated at West Virginia University Medical Center that illustrates the successful use of preoperative percutaneous angiographic embolization of a pheochromocytoma in controlling a malignant hypertensive crisis.

Intercostal muscle and myo-osseous flaps in difficult pediatric thoracic problems.

Intercostal muscle pedicle flaps have been successfully utilized in the treatment of recurrent tracheoesophageal fistulae and esophageal perforations in the pediatric age group. An intercostal myo-osseous pedicle flap was also created to repair a distal congenital tracheal stenosis at the carina. The viable intercostal flap has the advantage of multiplicity of uses and of considerable mobility. The presence of a blood supply assures healing. The option of retaining periosteum on the flap encourages bone regeneration at the site of tracheal or bronchial repair. The pleura of the flap provides an epithelial surface for intratracheal repair. The rib graft prevents stricture at the site of tracheal repair.

Advances in the management of gastroschisis.

Twenty-eight cases of gastroschisis have been treated over a five-year period. Twenty-two silos were placed and 19 infants had uncomplicated silo closure. Enlargement of the abdominal wall defect to allow optimum reduction of the edematous bowel was essential to closure in less than a week. Rapid removal of the prosthesis and strict adherence to aseptic technique prevented septic complications. Inability to return the bowel to the abdominal cavity within five to six days mandated re-exploration to determine the cause for failure to reduce the silo. Accordingly, three infants were re-explored. Two patients had unrecognized intestinal lesions and a third infant, whose defect had not been enlarged, had infarction of the midgut. Six infants underwent primary closure; two with preinatal evisceration and four who had concomminant cutaneous enterostomies performed for intestinal atresia. Intestinal atresia or stenosis occurred in 25% of these infants. Postoperative management was facilitated by insertion of a gastrostomy tube, early peripheral venous nutrition and later insertion of a central venous catheter for nutrition. The one postoperative death (3.5% mortality rate) resulted from failure to follow the principles of silo management as outlined in this report.

Idiopathic female intersex with clitoromegaly and urethral duplication.

Idiopathic female intersex presents in the neonate with characteristic anomalies of the external genitalia, complete and separate phallic and vaginal urethras and vesical outlet obstruction. Management of this disorder should initially focus upon adequate urinary drainage and proper sex assignment in the neonate. Successful management and surgical reconstruction of the external genitalia should later result in normal female development.