Hypospadias repair with the glanular-frenular collar (GFC) technique.

BACKGROUND: In the normal human penis, the glans wings merge in the midline ventrally, but are separated by the 'septum glandis' in conjunction with the frenulum. The frenulum is also included in the formation of the distal (glanular and subcoronal) urethra, which has a special part known as the 'fossa navicularis'. This has inspired a hypospadias repair technique that simulates the development of the glanular and subcoronal urethra, which can be incorporated into the repair of all cases of hypospadias. MATERIAL AND METHOD: A total of 121 patients with varying degrees of hypospadias underwent surgery with the described technique: a Y-V plasty was used to dissect the inner foreskin, in a fashion that allowed for its ventral mobilization as a frenular mucosal collar. After tubularization of the proximal urethra, a partial spongioplasty was performed that extended up to the subcoronal level. The glans wings were approximated only at their outermost convexities, with a couple of subepithelial sutures, leaving a slit for the meatus. The cleft-like area between the split wings of the glans penis was filled with the terminal ends of the spongiosum and the dartos of the mucosal collar, which converged to form a septum and a neo-frenulum (glanular-frenular collar, GFC). The midline skin closure of the ventral collar and the circumferential foreskin closure was completed as usual. RESULTS: At a mean follow-up of 10 months, two patients developed urethral fistula (2%), six had meatal stenosis (5%), and two had glans dehiscence (2%) that resulted in meatal retraction. Overall, patients had a cosmetically satisfying appearance (Figure). Forty-one received secondary circumcision; the parents of 80 (66%) patients were satisfied with the final foreskin appearance obtained with this method. DISCUSSION: The split wings of the glans penis or so-called ventral cleft between the glans wings that accommodate the frenulum is part of normal anatomy. Hence, in hypospadias surgery, the approximated glans wings should allow for ventral support of the glanular and subcoronal urethra through a reconstructed neo-frenulum. Neither glanular surface enhancement nor extensive dissection of the glans wings and their full-length approximation are necessary, and may in fact be counter-productive. CONCLUSIONS: The employment of a GFC provided: 1) an anatomical restoration of the distal (glanular and subcoronal) urethra, supported by a frenulum; 2) a protective (undissected) dartos layer over the distal part of the tubularized neourethra; and 3) a space for the re-formation of the fossa navicularis.

[FIRST WOMEN DOCTORS FROM TURKEY: AMALIA FRISCH (1882-1941)].

Amália Frisch was born in Edirne/Adrianople, Turkey, in 1882 to an immigrant Jewish family from Hungary. Following her graduation from the American College for Girls in Istanbul in 1901, she traveled to Switzerland for her medical education. Amália Frisch graduated from the school of medicine in Bern in 1907, and received her MD (Doctor universae medicinae) degree from the Zurich University in 1908. She specialised in gynaecology at the Vienna University Clincs, before returning to Istanbul. In the December of 1908, Dr. Amália Frisch was appointed intern to the Austro-Hungarian Hospital in Galata to attend the women's ward. During the Balkan Wars of 1912-13 the hospital housed the wounded of the Turkish Army in its Pancaldi premises, and Amália Frisch received medals of merit for her services both from the Habsburg Emperor Franz Josef and the Ottoman Sultan Mehmed Reschad V. Amália Frisch was an active member of the Ottoman Society for the Protection of Women's Rights (est. 1913). She was deported by the French occupation command in 1919 and returned to Budapest, after which she altered her profession to stomatology and dentistry. Amália Frisch passed away in Budapest during war, in 1941.

Disorders of sexual development in a cultural context.

BACKGROUND: Disorders of sexual development (DSD) are congenital conditions in which the development of the chromosomal, gonadal or anatomical sex can be deemed atypical. The external genitalia should appear 'normal' in size and shape from birth, with no question of abnormality, and the individual must receive appropriate social-environmental feedback in the course of the sexual maturation process. METHODS: We review regional differences in the variables considered important for gender assignment in individuals with DSD. Various approaches to certain forms of DSD are analysed within their cultural context. RESULTS: The decision to leave the sex of rearing undisturbed or to change it is difficult. It depends on the patient's age and the extent to which the gender identity has been established with parental gender preference, social, cultural and religious factors. Severe forms of genetically female congenital adrenal hyperplasia, androgen insensitivity syndrome, 17ß-hydroxysteroid dehydrogenase-3, 5α-reductase and cytochrome P450 oxidoreductase deficiencies are found to be the most difficult cases to diagnose and/or manage. CONCLUSION: Gender assignment in children with DSD is a subject of intense debate. Each case of DSD must be evaluated individually and on its merits and potentials. Although early admission and appropriate diagnostic facilities could provide the correct diagnosis, this is not the case in some cultures. It is seen that 'gender panic', social and religious concepts affect the decision-making process in gender assignment, especially in delayed cases.

Congenital pulmonary fibrosarcoma in a newborn with hypoglycemia and respiratory distress: case report.

Although primary bronchopulmonary fibrosarcoma is a rare tumor, it may be characterized by the symptoms of acute respiratory distress occurring during the first moments of life in a newborn. It is one of the leading congenital malignant neoplasms of the lung, but is considered a borderline tumor since its biological behavior is much more favorable than that of adult fibrosarcomas. In the absence of metastases, complete resection is curative. Histopathological diagnosis is not simple, as the microscopic characteristics may be confused with benign fibromatosis or malignant mesenchymal neoplasms. In this case report, we present a case of congenital pulmonary spindle cell tumor showing the features of fibrosarcoma, and we discuss the differential diagnosis of spindle cell lesions localized within the thorax.

The prenatal diagnosis of cloacal dysgenesis sequence in six cases: can the termination of pregnancy always be the first choice?

OBJECTIVE: Cloacal dysgenesis sequence is a lethal malformation, which usually requires termination. In this study, our aim was to evaluate the prenatal and postnatal diagnostic features of cloacal dysgenesis sequence and review the management of the patients. MATERIAL AND METHODS: The data of six cases of cloacal dysgenesis sequences were collected from the ultrasonography and neonatal records. The findings were evaluated in prenatal and postnatal periods. Chromosomal analysis was performed in all the cases. The evaluation of primary and secondary malformations was done. Coexisting anomalies were searched for by radiology and histopathology. RESULTS: Malformations in six cases (two females and four males) were described. The absence of anal, genital, and urinary openings with intact perineum covered by smooth skin were common findings. These features were considered as primary malformations for cloacal dysgenesis sequence. Secondary anomalies (urinary and gastrointestinal system malformations, pulmonary hypoplasia, and other coexisting anomalies) were evaluated. CONCLUSION: The prenatal differential diagnosis of cloacal dysgenesis sequence from other urinary obstructive diseases was essential regarding fetal prognosis, prenatal, and neonatal management. The bladder outlet obstruction and pulmonary hypoplasia due to reduced amniotic fluid and/or kidney disease were considered prognostic factors for neonatal death. Termination of pregnancy is almost always recommended instead of intrauterine shunt procedures; but if we take into consideration one of our cases and a few reported cases who survived in the neonatal period, the prenatal management of these pregnancies needs to be reevaluated.