Donald H. Klotz

Diagnosis and treatment of urethral prolapse in children

OBJECTIVES To review published reports on urethral prolapse in the pediatric population, with a focus on diagnosis and management, and to do a retrospective review of 20 cases of urethral prolapse at an urban hospital. METHODS A retrospective chart review of 20 consecutive cases of urethral prolapse in the pediatric population at Kings County Hospital was done. A review of the published reports on urethral prolapse from 1937 to the present was included in this study. RESULTS Twenty patients with urethral prolapse were treated at Kings County Hospital during a 10-year period. Patients were identified by perineal bleeding and diagnosed by physical examination. All patients were successfully treated by excision of the prolapsed urethral mucosa and suturing of the remaining mucosa to the vestibule. CONCLUSIONS Urethral prolapse is an uncommon entity that occurs primarily in prepubertal black girls. Patients may be successfully treated by excision of the prolapsed mucosa and suturing of the proximal urethra to the vestibule.

Pearls and perils in the management of prolonged, peculiar, penetrating esophageal foreign bodies in children

BACKGROUND/PURPOSE Most retained esophageal foreign bodies (FB) are identified soon after ingestion and are easily extracted. A minority of FB ingestions are not identified for weeks to years and present significant problems for retrieval. The purpose of this study was to describe the diagnostic and therapeutic strategies needed to care for children who have chronic esophageal FBs. METHODS Five children were identified as having retained esophageal FBs 2 months to 2 years after ingestion. During the same 3-year period, 100 children who had acute FBs were identified and had their foreign bodies removed endoscopically. The average age of the children was 3 years (range, 2.4 to 3.5). RESULTS The average age of the five children identified in this study was 3 years. The items ingested included coins, a heart pendant, a clothespin spring, and a toy soldier. Complications from chronically retained foreign bodies were bronchoesophageal fistula, mediastinitis, esophageal diverticulum, and lobar atelectasis. One patient died from an aortoesophageal fistula. In all children, endoscopic removal was attempted. Barium esophagram was then performed, and foreign bodies were eventually removed via right thoracotomy. CONCLUSIONS Long-retained esophageal FBs are extremely morbid and life threatening. History most often identifies excess salivation, new onset asthma, and/or recurrent upper respiratory infections. Three diagnostic adjuncts are helpful in identifying the presence of a long retained FB: (1) Chest x-ray (PA and lateral), (2) barium swallow, and (3) esophagoscopy. Indications for thoracotomy for removal of foreign body include (1) Poor endoscopic visualization of FB because of inflammatory tissue and (2) Herald bleeding during endoscopy.

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.

Choledochal cysts: Diagnostic and therapeutic problems*

Summary A review of eight patients with congenital biliary cysts showed that the correct diagnosis was only anticipated in two, that several patients underwent prolonged nonoperative treatment because of a mistaken diagnosis of hepatitis, and that even in patients with an abdominal mass the true character of the cyst was not suspected until a detailed radiographic work-up was completed. In contrast to previous publications, a review of our own cases and 138 cases reported during the last 4 yr indicates that choledochal cysts represent only one of various biliary cystic dilations that can occur in either an isolated or a multiple form. A review of operative procedures performed during the last decade reveals that excision and duct-enterostomy carry a mortality risk no higher than cyst-enterostomies, with fewer postoperative complications in a still-limited follow-up period.

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 class V patients. Infant (true) hermaphrodites in classes II 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.

Persisting Perfusion Defects After Bronchoscopic Removal or Spontaneous Expulsion of Aspirated Foreign Objects

Follow-up lung scans were obtained in 9 children to determine whether perfusion lung scanning could be used to verify completeness of foreign-body removal. In 4 patients, extensive perfusion defects were present 4-19 days after removal of the object. In 1 patient, the area of hypoperfusion was larger than the territory served by a persistently obstructed lobar bronchus. Follow-up lung scans are of limited value for verifying completeness of foreign-body removal and localization of retained fragments.

Surgical Therapy for Urethral Prolapse in Young Girls

Abstract Twenty black girls with urethral prolapse, aged 1½–8 years, were admitted over a 2-year period to the Pediatric Surgical Service that serves a predominantly black community. The most common finding consisted of a painless mass prolapsing through the urethral meatus, associated with bloody staining. Only four of these patients were referred to the Pediatric Surgical Service with the correct diagnosis. Incorrect diagnoses included sarcoma botryoides, hemangioma, urethral caruncle, ureterocele, trauma, alleged rape, or vaginal bleeding of unknown origin. History, physical findings and radiological work-up failed to reveal any identifiable cause for the urethral prolapse. Awareness of the common occurrence of urethral prolapse in black girls, prompt diagnosis, and operative treatment should limit hospitalization to 1 day without significant postoperative complications.