Choledochal cyst with perforation--an unusual presentation. Case report and review of the literature.

A unique case of a 22-month-old baby girl with a perforated choledochal cyst, who presented with vague abdominal symptoms but without any jaundice, an acute abdomen and an incidental finding of acholic stools, is described below with a review of the literature.

Imaging of uterovaginal anomalies in the pediatric patient.

Uterovaginal anomalies are relatively common congenital disorders whose diagnosis at a young age is important but often difficult. The various modalities currently employed for this evaluation are discussed, including MRI, the most recent available tool. The embryology of female reproductive tract anomalies is included.

Choledochal cyst and biliary atresia in the neonate: imaging findings in five cases.

The radiologic findings in five neonates with choledochal cyst associated with extra-hepatic biliary atresia are described. All five patients (age range, 13-72 days) presented with jaundice and acholic stools. In all four patients who underwent sonographic examination, a cystic structure separate from the gallbladder representing the choledochal cyst was shown. The diagnosis of atresia of the distal common bile duct was made preoperatively in all cases by hepatobiliary scintigraphy. Diagnosis was confirmed by surgical findings and was demonstrated by intraoperative cholangiography in four cases. All patients were successfully treated with surgical intervention within 1 month from the time of diagnosis. Early detection of this rare disorder, which may be distinct from choledochal cyst found in children and adults, is important to prevent fatal complications of biliary obstruction. The combined use of sonography and hepatobiliary scintigraphy can correctly identify this subset of patients with persistent neonatal jaundice and provide valuable information for prompt surgical management.

Results of levatorplasty for anal incontinence.

Since the introduction of the levatorplasty, 44 patients were operated upon for anal incontinence on our service. In 31 patients, a levatorplasty was performed. The selection of the levatorplasty as operative procedure was based on the availability of a functional levator as demonstrated by physical examination, anorectal manometry, and defecography. The 31 patients ranged from infancy to 76 years of age and 30 of them were followed up to 7 years with an average of 3 years. In 18 patients with fecal incontinence following operative procedures for anorectal malformations, satisfactory continence was restored in 8, improvement was found in 6, and 4 patients remain incontinent. In 13 patients with incontinence following operative procedures or trauma, the postoperative results were satisfactory in 9 patients, 2 improved, and 2 failed. The review showed that the levatorplasty was most successful in patients in whom the revised but normally innervated levator replaces a destroyed external sphincter in the presence of an intact internal sphincter. Only one half of all patients with major incontinence following an operative procedure for anorectal malformations had an adequate levator that allowed successful restoration of function. The review showed that the levatorplasty can improve or restore anal continence in a variety of conditions in properly selected patients.

Clavicular periostitis: an unusual complication of percutaneous subclavian venous catheterization.

The first three cases, to the authors' knowledge, of benign clavicular periostitis resulting from percutaneous subclavian venous catheterization in children and the mechanism of this iatrogenic trauma are discussed.

Posttraumatic splenic replantation in children.

Previous work in our laboratories showed that canine omental replantation of splenic wafers leads to reproducible viability, restoration of filtrative function, bacterial clearance, and normal antibody formation. An identical technique has been employed in children whose spleen could not be repaired since 1979. During a 3-yr follow-up viability of the replanted segments was documented with return of filtrative function, normal immunoglobulin, and antibody response. Splenic preservation through operative repair or selective nonoperative observation should remain the primary goal after splenic trauma. If this is not possible, splenic replantation represents an alternative approach in the treatment of splenic trauma.

Function of the replanted spleen in dogs.

The function of replanted splenic fragments was studied by comparing three groups of five dogs each, one group with intact spleens; one, post-splenectomy; and one with splenic replantation. Fifteen fragments were implanted into the omentum. Howell-Jolly bodies appeared after splenectomy but cleared in the replanted group after several months. I125-tagged attenuated pneumococcal clearance studies showed a significant difference between control and replanted group compared with the splenectomized group. The increase of pneumococcal antibody titers after vaccination differed significantly between the splenectomized and the replanted group. All replanted fragments were viable and showed growth over a 2-year period. These studies demonstrate that omental replantation of the canine spleen leads to the maintenance of certain functional splenic parameters comparable to the normal spleen which are significantly different from the splenectomized animal.

Absorptive function of the aganglionic ileum.

In children with colonic and iliac aganglionosis, the aganglionic intestine is usually either removed or bypassed. Since fluid absorption occurs predominantly in the terminal ileum, malabsorption often occurs unless the intestinal transit time is reduced. The Martin-Duhamel operation, reducing transit and thereby increasing the time of exposure of intestinal content to the lowest part of the ganglionic intestine, has been used successfully in most children with colonic and short ileal aganglionosis. With longer segments the slowdown of intestinal transit may be inadequate and the aganglionic ileum can then be used. The aganglionic ileum maintains its absorptive capacity if the obstructive element is removed using an aganglionic-ganglionic iliac anastomosis similar to the Martin-Duhamel technique.

Tongue lesions in children.

A review of tongue lesions in children showed that there is a great variety requiring operative intervention, often in infancy. While the presenting symptoms may be related to dysphagia and dyspnea, the aim of operative intervention should not only be to salvage life by restoration of breathing and swallowing, but also to leave a tongue capable of adequate speech, taste, sensation, and normal orofacial development. Intimate knowledge of lingual anatomy and function is necessary to allow selection of the ideal procedure and appropriate timing of the therapy. While careful observation and nonoperative approach may be indicated in non-neoplastic macroglossia, early intervention is often necessary in diffuse neoplastic lesions such as lymphangioma, fibromatosis, or fibrolipomatous dysplasia. While malignant tumors are rare in childhood, they do occur and have to ruled out.

The government and the inguinal hernia.

Our review of the incidence of inguinal hernias in children based on socioeconomic differences and their risk of incarceration with its subsequent complications, was prompted by a governmental decision to disallow elective herniorrhaphy in children over 1 yr of age. The review showed the following: The risk of incarceration, the failure of preoperative reduction and the potential gonadal injury is more than three times as high in the poor child, usually covered by Medicaid. Since operative and postoperative complications increase proportional to the incidence of incarcertain, the denial of an elective herniorrhaphy endangers the life of a child. Since the increased hospitalization after incarceration also increases the financial expenditure, this rule is not only medically but also economically unsound. This review suggests that unilateral governmental health care decisions, especially those aimed at cost containment, should be carefully scrutinized to determine what price not only the society, but the patient has to pay for the "cost containment". We feel that the price of the denial of a herniorrhaphy is too high.

Successful renal transplantation in infancy.

This infant's post renal transplantation course, representing apparently the smallest long term survivor, illustrates that neither age nor size are contraindications to successful renal transplantation in infants with end stage renal failure. Additional experience with the transplantation of a single kidney into a 1-yr-old baby weighing 4650 gm with congenital bilateral renal hypoplasia has also been successful with a 3-mo follow-up. Both cases demonstrate that single or double renal transplantation in infants is feasible and should be considered when indicated.

Traumatic death in urban children.

Trauma has remained one of the leading causes of death in children in spite of improved medical care. A review of 911 pediatric trauma deaths which occurred over a 5 yr period in an urban setting revealed that almost 50% of these children died before receiving medical care. A significant improvement of the trauma mortality can thus only be accomplished by reducing the number of "DOAs". We therefore analyzed the cause and type of injury and its relationship to age, sex, race, seasonal occurrence, and sociological circumstances. The following four categories are merely part of the overall material developed in this review. Even with improved medical care of trauma patients the overall pediatric trauma mortality cannot be significantly reduced unless the number of DOAs is decreased through prevention. Educational and family assistance programs can be designed for specific problem areas to reach identified susceptible groups through existing channels such as day care centers, schools, or welfare agencies. Statistical data, such as presented here (but not previously available) are essential to analyze the particular problems of specific geographic and sociologic areas. Since the vast majority of pediatric trauma deaths fall within the interest sphere of the pediatric surgeon, our active participation in accident prevention is essential to achieve a significant reduction of pediatric trauma mortalities.

Familial juvenile adenomatous polyposis.

Most colonic polyps in children are of the juvenile type and occur either as single or scattered colonic polyps. The peak incidence occurs between 4 and 6 yr of age, with a spontaneous decline from 12 to 15 yr. Significant clinical symptoms are rare, and operative therapy is rarely indicated. Diffuse colonic juvenile polyposis, however, varies with different clinical, prognostic, and genetic implications. In infancy, colonic polyposis may be associated with diffuse gastrointestinal involvement leading to fatal complications unless treated aggressively. In childhood, colonic polyposis can occur with a genetic variance with an increased incidence of familial intestinal malignancies. Colonic polyposis in childhood, both familial and nonfamilial, can present with a mixed form of juvenile and adenomatous polyposis. In children with colonic polyposis, the biopsy of a single polyp that reveals the histologic appearance of a juvenile polyp does not rule out the simultaneous existence of adenomatous polyps.