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Mayer Rokitansky Kuster Hauser Syndrome – Developmental Aspects and Psychological Effects




MRKHS is a congenital disease that affects women and the sexual reproduction and the elimination process. The developmental psychological issue is the sexual reproductive state and the issue of feeling normal and comfortable during the developmental phases into adulthood and through elder age. MRKHS can impact the female reproductive system through the absence of the vagina and uterus (Burel et al). The absence of the female primary genitals and reproductive function can cause an underlying inferiority complex that is linked to the “Disorder of Sex Development (DS),” (Bean et al). The inferiority complex is additionally linked to the quality of life the condition creates. “A young woman’s sense of well-being and quality of life are impacted by the condition,” (Bean et al). The well-being of the young woman is a condition that directly impacts the developmental psychology.

Syndrome Research

The physical limitations give rise to female sexual inadequacy and reproductive dysfunction. The reproductive dysfunction is in the lack of “fertility and the physical capacity to mate,” (Wallen & Zehr, p. 101). The developmental concerns however do not generally receive the public health focus the condition should receive (Lerner, Easterbrooks and Mistry, p. 314). The psychosocial aspect is from the biological standpoint that impacts the female “biology-person-sociocultural relational matrix,” (Lerner et al, p. 37). The psychological impact is a function of the development of neurochemical brain processes that link the identity of self to well-being (Lerner et al). The impact is with respect to the understanding of the female sexual reproduction and attached sense of value from the “centrality of embodiment for a cultural psychology,” (Lerner et al, p. 37).

Women generally do show normal development of secondary sexual characteristics that identify as female in public situations. The normal development of the secondary sexual characteristics however does hide the abnormal sexual development of the primary sexual characteristic and abnormal reproductive development. The MRKH syndrome is characteristic of genetic defects linked to the autosomal dominant trait that “suggests the involvement of either mutations in a major developmental gene or a limited chromosomal imbalance,” (Morcel et al, p. 2). This genetic link establishes the syndrome as a condition affecting physical development from a young age and impacting the psychosocial development of the young woman during adolescence.

Cognitive impairment from the psychosocial impact of MRKH syndrome is most readily linked to the inferiority complex. The complex is not however linked to the lack of external development of the primary sex characteristics as the vagina from the outward appearance is developed. The impact is by the lack of internal development of the vagina and the lack of uterine development (Morcel et al, p. 2-13). The cognitive impact is generally with the development of personal relationships and the physiological development of sexual relationships and the process of maturation. The developmental psychological issue extends to the maturation of the female and the choice to pursue sexual relationships. “The independence between hormonal state and sexual behavior allows sex to be used for social purpose,” (Wallen & Zehr, p. 101).

Further analyses by Wallen & Zehr establish the following: This dependence upon psychological mechanisms to coordinate physiology with behavior is possibly unique to primates, including humans, and allows a variety of nonphysiological influences, particularly social context to regulate sexual behavior (p. 101).

Sexual behavioral patterns, which can be dictate based on psychosocial conditioning and cognitive development, can be impacted by the abnormal developmental psychology from the underlying syndrome. The specifics of the underdevelopment are conditional to the genetic factors that enable the syndrome. The disorder is with the development “of the Mullerian ducts occurring between the 4th and 12th week of gestation. The syndrome is described as associated with congenital spinal anomalies,” (Strubbe et al, p. 460). From the genetic standpoint the underdevelopment during the gestation period will impact the cognitive psychosocial development of the female from the time there is an understanding of the syndrome and its impact on the sexual and reproductive functions.

The difference between the sexual and reproductive functions of the syndrome is based on the impact from the lack of vaginal development and lack of uterine development. The ovaries however do develop and however the ovaries can only generate eggs and are seemingly unable to enable the continuation to the process of gestation. The overall health impact from the syndrome however is very associative to impairment of health functions that affect the sense of self. “The most common coexisting defects affect the upper urinary tract, including unilateral renal agenesis, ectopia of one or both kidneys, renal hypoplasia, horseshoe kidney and hydronephrosis,” (Bernardini et al). The Mullerian aplasia, Mullerian agenesis and MRKH syndrome all refer to the “vaginal agenesis is the congenital absence of the uterus and vagina,” (Burel et al).

Further physical abnormalities exist outside of the sexual reproduction area. “The skeletal abnormalities reported are fusion anomaly of vertebrae, congenital scoliosis, and limb deformities like brachymesophalangy of digits, small distal phalanx of digits, long proximal phalanx of digits, and long metacarpals of digits,” (Gupta & Ansari, p. 111). The spinal condition is linked to the “incidence of spina bifida occulta,” (Strubbe et al, p. 461). The psychosocial impact can be derived from the condition of scoliosis, which is the “massive congenital fusion of vertebrae, as normal spinal development is not possible where there are multiple fusions of spinous processes, laminae, and vertebral bodies,” (Strubbe et al, p. 461).

The specific development psychological impact from MRKH syndrome is identified in the clinical standpoint of psychological distress (Morcel et al, p. 2-13). “Young women diagnosed with MRKH syndrome suffer from extreme anxiety and very high psychological distress when they are told they have no uterus and vagina,” (Morcel et al, p. 2-13). The recommendation to enable proper psychosocial development is to engage the patient and family members in counseling treatment prior to the diagnosis and thereafter (Morcel et al, p. 2-13). The condition signals the comprehensive focus on cognitive development based on the identification of physiological abnormalities related to the underlying condition and from coexisting defects and secondary conditions linked to the syndrome.

Further issues include the potential for hearing impairment and heart abnormalities (Morcel et al). The similarities between the MRKH syndrome and associated disorders establish the basis for the association to the anomalies that can occur. The condition is often linked to the renal and skeletal abnormalities and thus is characteristic of physical abnormalities with an exterior and interior basis (Morcel et al). The psychosocial basis for cognitive development of normal psychological growth is reflective of the underlying physical developmental abnormalities and the problems arising with self-identity and the inferiority complex.

The patient with the MRKH syndrome diagnosis will face the condition from an early age and will have to face medical maintenance issues that can impact their life well into the future. The psychological developmental issues are with due to these cognitive development issues as time passes from childhood through adolescence and into adulthood. Counseling will likely be only part of the underlying alteration to apply toward changing psychosocial manifestations based on the impact from society onto the psychosocial development. The complexity of the syndrome can also frighten the patient well beyond the level of anxiety and could potentially lead to episodes of psychosis should the underlying coping mechanism fail to adjust the patient to accept the condition.

Normalcy is part of the identity factor to establish psychosocial acceptance for cognitive development. Developmental psychological deficiencies will stem from the lack of normalcy as a sense of well-being relative to the health maintenance required by the syndrome. As the patient ages, the impact of perhaps not feeling ‘womanly’ or as a normal female can generate cognitive antisocial behaviors as a path of neuropsychology. The patient could experience withdrawal symptoms and can manifest itself as introvert behavior, which will link to antisocial behavior. The impact of antisocial behavior would extend from the anxiety associated with MRKH syndrome and the underlying physical condition. The antisocial behavior can worsen based on the comprehensive nature of the afflictions associated with the condition.

The psychosocial impact can also reflect the underlying sexual development during the adolescent phase. Although secondary sexual characteristics are developing, the lack of vaginal development and the potential for scoliosis and renal problems can identify the female as malformed and can disenable the impact from any earlier counseling sessions. The developmental psychological impact is therefore an issue that can trigger disruptions in psychosocial development leading to abnormal psychosocial behavior. The identified abnormality in behavior has been identified as antisocial but can also lead to further neurological disorders such as obsessive compulsive disorder or mental depression and the associated impact of melancholy.

The hormonal balance/imbalance for females also is critical to the underlying developmental psychology. The ovulatory menstrual cycle can lead to depression for the female given the potential for the lack of a period during menstruation and the inability to mate when the desire for mating is increased by hormonal responses (Wallen & Zehr). The malformation of upper extremities and congenital anomalies associated with the cervical spine are perhaps the most apparent physical abnormalities that are readily linked to the feeling of the inferiority complex. The inferiority complex can identify the basis for the neuropathology linked to the developmental psychology of the patient. The developmental aspects are psychomotor developmental with respect to the underlying responses associated with the coping mechanism during instances that test the female psychosocial response.

Overall, the patient is developing a coping mechanism to address the abnormal physiological development of the primary genitalia. The cognitive development is impacted by means of psychosocial identification of the female among other members of the gender given the parameters that establish the feelings linked to the inferiority complex. The female must identify as female when the physical body is unable to address the needs of the sexual relationship and the needs of the reproductive relationship. The inability to identify with the female gender in a sexual or reproductive manner can ostracize the patient and create a wall that will inhibit psychosocial development through counseling sessions. This inability can impact the developmental psychology of the patient throughout their life. If further impairment develops, the patient can further experience anxiety and the lack of interest in social development. The continuation of antisocial behavior can manifest into psychopathy that cannot be readily addressed through counseling. The lack of association with society will enable disassociation from relationships with either gender, as the female is unable to relate to the feminine aspects of women and is unable to address the sexual or reproductive needs and desires of the male gender.

References

Bean, et al. Mayer-Rokitansky-Kuster-Hauser Syndrome: Sexuality, Psychological Effects, and Quality of Life. Journal of Pediatric Adolescent Gynecology. Vol. 22, pp. 339-346.

Bernardini, L., et al. Recurrent microdeletion at 17q12 as a cause of Mayer-Rokitansky-Kuster-Hauser syndrome: two case reports. Orphanet Journal of Rare Diseases.

Burel, A., et al. Role of HOXA7 to HOXA13 and PBX1 genes in various forms of MRKH syndrome (congenital absence of uterus and vagina). Journal of Negative Results in Biomedicine.

Gupta, M., Kharb, V. MRKH Syndrome: Psychological Disturbances and Suicide. Journal of Indian Academy Forensic Medicine. Vol. 34. No. 1. ISSN 0971-0973.

Lerner, R., Eaterbrooks, M., Mistry, J. Handbook of Psychology, Developmental Psychology. Vol. 6. John Wiley & Sons. New Jersey. Print.

Morcel, K., Camborieux, L. Mayer-Rokitansky-Kuster-Hauser syndrome. Orphanet Journal of Rare Diseases. Vol. 2: 13.

Strubbe, E.H., et al. Spinal abnormalities and the atypical form of the Mayer-Rokitansky-Kuster-Hauser syndrome. Skeletal Radiology. Vol. 21, pp 459-462.

Wallen, K., Zehr, J.L.. Hormones and History: The Evolution and Development of Primate Sexuality. Journal of Sex Research. Vol. 41, (1)