[An infant of neurofibromatosis with external genitalia involvement presentation]

The vulva encompasses the external structures of the female reproductive system. At the forefront is the monpubis, a cushion of fatty tissue atop the pubic bone. Once puberty sets in, this area typically grows hair. Extending from just behind the mons pubis are the labia majora (labia = 'lips'; majora = 'larger'), which are larger skin fs olds coated with hair. Nestled within are the labia minora (labia = 'lips'; minora = 'smaller'), which are thinner, more...

Neurulation is the embryological process which forms the precursors of the central nervous system and occurs after gastrulation has established the three primary cell layers of the embryo: ectoderm, mesoderm, and endoderm. In humans, the majority of this system is formed via primary neurulation, in which the central portion of the ectoderm—originally appearing as a flat sheet of cells—folds upwards and inwards, sealing off to form a hollow neural tube. As development proceeds, the...

Like autosomes, sex chromosomes contain a variety of genes necessary for normal body function. When a mutation in one of these genes results in biological deficits, the disorder is considered sex-linked.

Development of the reproductive organs in an embryo starts from a bipotential state. This means the early embryo can develop either male or female reproductive organs. The formation of these organs begins with the growth of gonadal ridges that arise from the intermediate mesoderm during the fifth week of development.
Near the gonadal ridges, two duct systems are present: the mesonephric ducts (Wolffian ducts) and paramesonephric ducts (Müllerian ducts). These ducts form the basis for the...