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Biomedical subjects

D H Klotz

Publications and source records attributed to D H Klotz.

16 recordsLinked to original sources

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.

Child↗

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.

Abnormalities, Multiple↗

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.

Child↗

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.

Anal Canal↗

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.

Adolescent↗

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.

Female↗

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.

Adolescent↗

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.

Child↗

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.

Adolescent↗

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.

Airway Obstruction↗

Mediastinal esophageal duplication cyst associated with a partial pericardial defect.

A two-year-old girl with a mediastinal esophageal duplication cyst is the twelfth example of a thoracic cyst of foregut origin or pulmonary "sequestration" (bronchopulmonary foregut malformation according to the expanded definition of Gerle et al.) that has been associated with a partial pericardial defect on the same side, suggesting that anomalous thoracic masses of several morphologic types have a similar pathogenesis.

Bronchopulmonary Sequestration↗