Perforation of the ileum secondary to Enterobius vermicularis report of a rare case.

An isolated ileal perforation is presented in a 12-yr-old patient. Microscopic examination revealed granulomatous inflammation due to Enterobius vermicularis (pinworm). This is only the third documented report of intestinal perforation involving pinworms. Although infestation with this parasite is generally considered to be relatively harmless, on rare occasions, it can be a significant cause of morbidity due to its potential ability to invade the tissues.

Utero vaginal malformations: a trap for the unsuspecting surgeon.

Eighteen girls with major uterovaginal malformations were admitted to the Pediatric Surgical Service over a 17-year period. The diagnosis was not suspected or delayed in more than one half of the patients. The encountered anomalies were divided into four groups: I, isolated uterovaginal malformations (UVM) (4); II, UVM with anorectal anomalies (8); III, UVM with cloacal (urogenital sinus) abnormalities (5); and IV, caudal twinning (1). Imperforate hymen, vaginal web, low vaginal obstruction, or disorders of gonadal or chromosomal development were excluded. Patients presented with an abdominal mass or distension (5), abdominal pain (4), "sciatic"-like pain (1), purulent vaginal discharge with perineal pain (1), amenorrhea (2), and a pelvic and prerectal mass (1). The introitus was reported as normal in 11 patients with vaginal atresia or agenesis by the primary physician. Diagnostic studies, in addition to clinical and endoscopic examination, included routine radiological workup, genitourinary contrast studies, pelvic and perineal sonography, computerized tomography (CT) scanning, and more recently, magnetic resonance imaging. In complicated UVM, especially vaginal duplications with unilateral atresia, the CT scan was the most helpful diagnostic tool. Laparotomy was necessary, not only for therapeutic, but diagnostic reasons; even so, complex anomalies, such as vaginal duplication with unilateral atresia and a septate uterus, could not be suspected. Treatment was directed toward the restoration of a functional uterovaginal tract and the frequently associated anorectal anomalies. A review indicated that contrary to our expectations, the major UVM occurred in children with a low imperforate anus rather than the high variety.

Early gender assignment in true hermaphroditism.

True hermaphroditism is a rare form of intersex anomaly where both male and female gonads are present. True hermaphrodites diagnosed in the past have been preferentially reared as males. However, based on the appearance of their external genitalia and the ability to reconstruct a functional genitourinary system, most of these children should be raised as girls. Between 1968 and 1987, we managed eight children with true hermaphroditism. The karyotypes were as follows: five 46XX, one 46XY, one 47XXY, and one 46XX/mosaic. Gonadal distribution was bilateral (two ovotestes) in two, lateral (testis and ovary) in two, and unilateral (ovotestis and ovary) in four. Seven patients were diagnosed shortly after birth. Six were raised as girls, two as boys. Based on a review of the relationship of external-internal genitalia, gonads, and karyotype of our own patients and those published by others, we believe that the appearance of the external genitalia alone allows a prompt and definitive gender assignment in infancy. This led us to develop a classification of external genitalia based on the following characteristics. Class I represents normal female genitalia, class II an enlarged clitoris, class III partially fused labioscrotal folds, class IV fused labioscrotal folds, class V a hypoplastic scrotum and penoscrotal hypospadias, and class VI represents normal male genitalia. We had two patients that were class II, two class III, three that were class IV, and one to IV should be raised as females; in class V, male gender assignment is preferable. This classification, based on the appearance of the external genitalia, allows a simple screening and prompt gender assignment in infants identified as true hermaphrodites.

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.

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.

Absence and retention of small bowel gas in infants with midgut volvulus: mechanisms and significance.

The radiographic pattern of high intestinal obstruction--gastric and duodenal distension with absence of small bowel gas (ASBG) or a completely gasless abdomen--may be present in infants with midgut volvulus. In most instances, ASBG is associated with viable small bowel. However, when associated with abdominal distension and tenderness ASBG usually reflects strangulated midgut volvulus and bowel necrosis. In some patients with midgut volvulus there is a radiographic pattern of low small bowel obstruction; this is generally associated with necrotic bowel. These seemingly opposite observations can be explained by reviewing the dynamics of gas absorption in normal bowel and in closed loop intestinal obstruction. Impaired blood supply can result in either gaslessness or gaseous distension under differing conditions. Key words: Infant, intestinal tract, intestinal gas, intestinal obstruction, intestinal volvulus, malrotation, gasless abdomen.