Female circumcision.

Female circumcision, more accurately known as female genital mutilation, is still a common practice in parts of Africa. This ritual genital operation can involve partial or complete excision of the clitoris and labia minora as well as infibulation (labial fusion). The case reported here involves a 16-year-old African girl with a complication of this mutilating procedure.

Penoplasty for buried penis in children: report of 50 cases.

PURPOSE: The authors report their experience with a large number of children with buried penis and describe their surgical technique. METHODS: Fifty consecutive cases of buried penis that occurred between 1993 and 1999 were analyzed by chart review. Age at the time of surgery was 11 months to 13 years. Each patient underwent elective surgical repair utilizing a surgical technique not described previously. The authors' technique avoids a circumferential incision at the base of the penis, decreasing postoperative edema. The authors also utilize a unique through and through vertical mattress suture at the base of the penis that firmly attaches the shaft skin to the underlying corpora. RESULTS: All patients had a good to excellent outcome with a low postoperative complication rate. One patient fell during the postoperative period and developed a wound dehiscence. Early in the series 3 patients underwent additional procedures for recurrent retraction (6%). All patients experienced some postoperative discomfort as expected. Edema, when present, was mild and resolved within an acceptable period of time. All patients had a good cosmetic result with increased visualization of the penile shaft. CONCLUSIONS: A surgical approach to the buried penis is warranted in most circumstances. There are psychological benefits to both the patients and the parents. Although the authors perform the procedure as early as 11 months, it can be performed safely at 3 months. The procedure reported here provides immediate excellent cosmetic results with a low complication rate. The authors do not recommend suprapubic lipectomy either alone or in combination with the buried penis procedure. Circumcision should be avoided in an infant with a definite diagnosis of buried penis.

Genitourinary injuries in the newborn.

BACKGROUND: Circumcisions and cesarian sections are common procedures. Although complications to the newborn child fortunately are rare, it is important to emphasize the potential significance of this problem and its frequent iatrogenic etiology. The authors present 7 cases of genitourinary trauma in newborns, including surgical management and follow-up. METHODS: The authors relate 7 recent cases of genitourinary trauma in newborns from a children's hospital in a major metropolitan area. RESULTS: Case 1 and 2: Two infants suffered degloving injuries to both the prepuce and penile shaft from a Gomco clamp. Successful full-thickness skin grafting using the previously excised foreskin was used in 1 child. Case 3, 4, and 5: A Mogen clamp caused glans injuries in 3 infants. In 2, hemorrhage from the severed glans was controlled with topical epinephrine; the glans healed with a flattened appearance. Another infant sustained a laceration ventrally, requiring a delayed modified meatal advancement glanoplasty to correct the injury. Case 6: A male infant suffered a ventral slit and division of the ventral urethra before placement of a Gomco clamp. Formal hypospadias repair was required. Case 7: An emergent cesarean section resulted in a grade 4-perineal laceration in a female infant. The vaginal tear caused by the surgeon's finger, extended up to the posterior insertion of the cervix and into the rectum. The infant successfully underwent an emergent multilayered repair. CONCLUSIONS: Genitourinary trauma in the newborn is rare but often necessitates significant surgical intervention. Circumcision often is the causative event. There has been only 1 prior report of a perineal injury similar to case 7, with a fatal outcome.

Horse-related injuries in pediatric patients.

PURPOSE: The aim of this study was to describe the characteristics, nature, severity and outcome of injuries from horse-related trauma in pediatric patients, aged of 19 years or younger. METHODS: Retrospective analysis was conducted of 315 patients recorded in the National Pediatric Trauma Registry from February 1995 to August 1999. RESULTS: A total of 62% of the 315 patients were girls. The median age of injury was 10 years. Sixty-five percent of the patients were injured while mounted on a horse, and the most common mechanism of injury was falling off the horse. The most frequent reason for hospital admission was skeletal fractures followed by head injuries. The head, neck, and face area was the most commonly injured anatomic site, followed by the upper extremity, the abdomen, and then the lower extremity. The median length of stay in the hospital was 2 days. Forty percent of the patients needed treatment in the intensive care unit with a median length of stay of 2 days. Thirty-nine percent of patients underwent surgical procedures. The Injury Severity Score ranged from moderate to critical in 31.5% of the children. There were 8 deaths, 2.5% of the injured children. The most common cause of mortality was head injuries. Of the 307 survivors, 3% were discharged to a rehabilitation center, and 2% of the children had 1 or more functional impairments lasting longer than 7 months after discharge. CONCLUSIONS: Horse-related trauma is frequent in children and can cause severe injuries resulting in death and long-term disability. Awareness of the nature of injuries is important to avoid underestimation of their severity.

Rotary atlanto-axial subluxation with torticollis following central-venous catheter insertion.

Atlanto-axial subluxation with torticollis is an uncommon condition that occurs in children usually as a result of pharyngeal infection, minor trauma, or neck surgery. Passive motion of the head and neck during general anesthesia is probably another etiologic factor. Torticollis is the most common presenting physical finding. Pain may or may not be present, but is commonly present with passive neck motion. Neurologic sequelae are uncommon. Our case illustrates this condition as a complication of central venous catheter (CVC) insertion in a child under general anesthesia. The surgeon should suspect this pathology when a child presents with torticollis following CVC placement. Precautions should be taken in the operating room to avoid aggressive rotation and extension of the child's neck while under general anesthesia whether or not cervical inflammation is present. Special attention to head and neck positioning should be taken in patients with Down's syndrome since they are at increased risk for atlanto-axial subluxation. The prognosis is excellent when diagnosed early. A delay in diagnosis can result in the need for surgical intervention.

Cremasteric muscle hypertrophy accompanies inguinal hernias in children.

PURPOSE: The physical finding of a thickened spermatic cord as indicator of an inguinal hernia in children is well documented. This thickening has been attributed to the presence of a hernia sac. Based on our clinical observations, cremasteric fibers appeared thickened in the presence of an inguinal hernia. The authors postulated that hypertrophy of cremasteric fibers contributed to the physical finding of cord thickening. METHODS: The authors evaluated 18 patients ages 5 months to 9 years, 17 boys and one girl. Each patient was found to have a unilateral inguinal hernia with palpable thickened cord on the affected side. The contralateral side contained no detectable hernia or palpable cord thickening. Each patient underwent repair of the clinical hernia and exploration of the contralateral groin. Cremasteric fiber biopsy sections were taken from both sides for cross-sectional measurement and comparison. The biopsy specimens were evaluated by a pathologist unaware of the hypothesis. RESULTS: In 15 of 18 patients microscopic measurement of the cremasteric fibers showed hypertrophy on the side of the clinical hernia when compared with the contralateral side. The contralateral side was found to have either no hernia or a small patent or obliterated processus vaginalis. CONCLUSION: The authors conclude that the palpable thickening of the spermatic cord, the "silk glove sign," in most cases is caused not only by an empty hernia sac but also by hypertrophied cremasteric muscle fibers.

Long-term remission of Crohn's disease treated with thalidomide: a seminal case report.

A 31-year-old female with severe Crohn's disease for 15 years who had been treated with corticosteroids and 6-mercaptopurine, was treated with thalidomide initially for erythema nodosum. While on thalidomide all symptoms of Crohn's disease disappeared and she was able to discontinue all other drugs. At this writing she has been on thalidomide as sole therapy for over 4 years with the exception of a 5-week hiatus, during which time her symptoms recurred, but again disappeared after resumption of thalidomide therapy. This case suggests that thalidomide may be a useful therapy for Crohn's disease and provides impetus for a clinical trial of thalidomide for Crohn's disease.

Neonatal rectovaginal tear during cesarean section.

Intrauterine rectovaginal tears are an infrequent and life-threatening complication of breech presentation. One previous case has been presented in the literature with a fatal outcome. The authors report a second case of a severe rectovaginal intrauterine tear sustained during cesarean delivery. Prompt administration of antibiotics followed by debridement and primary repair was performed with a successful outcome.

Rectal duplications.

Recent experience with two cases of rectal duplication, which had been misdiagnosed as hemorrhoids, or fistula-in-ano with resultant delay in diagnosis, prompted us to review our prior experience with 11 of these unusual cases. Age at presentation ranged from newborn to 18 years (mean, 17 months). The most common presenting sign was a perianal or anal fistula, observed in five children. Two children presenting with fistulae had concomitant infection in the duplication. Other presenting signs included obstruction or prolapse caused by the rectal mass in three patients, rectal bleeding in three, and urinary retention in one. Some children presented with more than one finding. No associated spinal or vertebral anomalies were observed. Total excision was performed using a transanal approach in eight patients, postanal (transcoccygeal) in two, and posterior sagittal in one. Postoperative continence was normal in all patients. These cases illustrate that rectal duplications can be confused with other types of anorectal pathology including hemorrhoids, fistula-in-ano, and perirectal abscess. Total excision performed using a posterior sagittal, transanal, or transcoccygeal approach is curative.

Subsequent asynchronous torsion of normal adnexa in children.

Children who have suffered from ovarian torsion may be at increased risk for a repetitive event. Torsion in a normal adnexa may be due to excessive mobility resulting from congenitally long supportive ligaments. Oophoropexy or shortening of the ligamentous support of the remaining functional ovary after torsion is recommended in attempt to prevent a subsequent torsion. The evaluation of young females with abdominal pain should always include the consideration of ovarian torsion. Preoperative ultrasonography is not invasive and could lead to earlier operative intervention resulting in salvage of ovarian tissue. Observation in these same children may allow a torsed edematous ovary to convert to a nonviable necrotic tissue necessitating oophorectomy. Laparoscopy is useful in cases in which the diagnosis is unclear.

Pediatric lateral ventral (spigelian) hernias.

Lateral ventral (spigelian) hernias may not be recognized if the physician is unaware that they can occur in pediatric patients. Diagnosis depends on finding a protrusion in the spigelian fascia lateral to the rectus sheath at the junction of the arcuate and semilunar lines, below the umbilicus. We report nine such cases, to bring the total number of recorded pediatric cases to 18. Surgical repair involves a layered overlapping closure using interrupted nonabsorbable sutures. Since the rim of the defect is extremely difficult to outline once the child is asleep and relaxed, it must be outlined in indelible ink while the child is awake and straining.

Bilateral neonatal torsion.

Extravaginal neonatal torsion of the spermatic cord, although rare, is being recognized with increasing frequency. Bilateral neonatal torsion is even more uncommon. Only 1 previous case of asynchronous neonatal torsion has been reported. Controversy exists concerning the urgency of exploration as well as the need for contralateral orchiopexy, since some reports suggest that the contralateral testicle is not at risk for torsion. We present 4 consecutive cases of bilateral neonatal torsion seen between 1966 and 1986. Two cases were asynchronous, 1 of which with a previously normal-appearing contralateral gonad was rendered anorchid. In the other patient a testicle was salvaged by prompt intervention. We have adopted a policy of prompt exploration with contralateral orchiopexy in all cases of unilateral neonatal torsion. Treatment of the ipsilateral testicle is determined by operative findings.

Bladder duplication with one exstrophy and one cloaca.

Bladder duplication is a rare anomaly. Less than 100 cases of all types have been described in the literature. When duplication of the bladder occurs, it has previously been described as a mirror image or as a septate bladder. A newborn female presented with what appeared to be a complete bladder exstrophy, a large ruptured omphalocele with perforated large bowel, an imperforate anus, and a bifid clitoris. Urine was seen emanating from various sites: the bladder mucosa, the mucous fistula constructed at the time of the colostomy, and the left hemiclitoris. The anatomic puzzle was resolved when multiple studies disclosed the problem to be a bladder duplication. The bladder on the anterior abdominal wall was exstrophied, the intraabdominal bladder was a cloaca, and the left hemiclitoris contained a phallic urethra, which drained the intraabdominal bladder. Total correction with normal function was obtained. This patient demonstrates that bladder duplication may present in various ways.

A technique for radiographic cannulation and opacification of anatomical structures with small orifices--a new method.

A technique for the injection of contrast material into structures with small orifices which cannot be catheterized in the usual manner is described and illustrated. A small occlusion balloon catheter (Medi-Tech, Watertown, MA) allows for orifice cannulation and occlusion for retrograde contrast examination.

To pex or not to pex.

There is little agreement as to the performance of an exploration and orchiopexy on the asymptomatic side when the neonate is found to have unilateral extravaginal torsion of the testis. If extravaginal torsion does not present bilaterally, many surgeons feel that the uninvolved testis is not at risk. Extravaginal torsion of the testis, fortunately, is a rare problem. Armed with the knowledge of this case report and one previous case report, however, it is clear that extravaginal torsion can present as separate and asynchronous events. Knowledge of this fact should stimulate surgeons to explore and pex the contralateral side upon finding extravaginal torsion of the testis.

Persistent incompetence of the cervix in female infants with congenital abnormalities of the pelvic viscera and perineum.

Vaginal reflux during voiding cystourethrography (VCUG) is a common finding in young females. Reflux of urine into the uterus, fallopian tubes, and peritoneal cavity documented by contrast studies is rare and considered abnormal. Chronic retrograde flow of urine and its contaminants through an incompetent cervical os mechanism into the peritoneal cavity may lead to peritonitis, pelvic inflammatory disease, and sterility. Five females with congenital abnormalities of the pelvic viscera and perineum had reflux into the uterus, fallopian tubes, and/or peritoneal cavity during voiding studies done before or months to years after successful corrective surgery. The findings were compatible with a persistent incompetent cervical os. It appears that this group of patients is at risk for the development of chronic pelvic inflammatory disease and its consequences.

Ambulatory serial excision of giant nevi.

Nevi over the size of a dime at the time of birth should probably be removed. Nevi this size often cannot be differentiated histologically from the true giant nevus that may cover over 50% of the body. Giant nevi have a 2%-30% malignant potential. In order to avoid the morbidity of split-thickness skin grafts when removing the larger nevi, we have been successful in serially excising many nevi as ambulatory procedures. Most moderately large nevi, regardless of their location, can be managed with this method.