scholarly journals Congenital Megalourethra Associated With Urethral Duplication: A Case Report

2020 ◽  
Vol 19 (1) ◽  
pp. 53-56
Author(s):  
Muhammad Humayun Kabir ◽  
Tanvir Ahmed ◽  
Md Saiful Islam ◽  
SM Yunus Ali ◽  
Md Rafiqul Islam

A 28 years old male with well developed secondary sexual characteristics was admitted in BSMMU with a grossly swollen, malformed phallus and passage of urine mostly through an opening in the perineum. After birth, his parents noticed that when the baby micturates the phallus is swollen and few drops of urine comes out through the external urethral meatus but most of the urine comes out through an opening in the perineum. No treatment was taken by his parents for this complaint. At puberty his secondary sexual characteristics developed normally. He felt sexual urge towards females but his penis was not erected. At the peak of his sexual excitement, orgasm occurs with release of whitish seminal fluid which comes mostly through perineal opening. His general physical examination was unremarkable & examination of the genitalia revealed well developed scrotum. Both of the testes were normal in size, shape and consistency but the penile shaft was large and flabby, and the ventral aspect of the penis appeared as a sac with mild pseudo phimosis. Careful palpation suggested the absence of the corpus spongiosum & corpora cavernosa. Both these findings were subsequently confirmed by a duplex colour doppler study. The penis distended ventrally when he tried to micturate and the urinary stream was narrow. About 75% of urine flow evacuates through perineal opening which was located about 2 cm from anal opening in the midline ventrally. A voiding cystourethrogram revealed that whole of the anterior urethra was grossly dilated with narrowing at the bulbar part & external urerthral meatus. There was another passage (about 5 cm) from the scrotal margin up to proximal posterior urethra. Cystogram showed a bladder diverticulum at left lateral wall. An urethrocystoscopic examination revealed that just proximal to the stenosed external urethral meatus the urethra is widely dilated which seemed like a transparent fusiform sac with irrigating fluid. Whole of the anterior urethra was very thin walled with transmitting lights through it. Proximal part of the urethra near the external sphincter was grossly narrowed. Urethrocystoscope was also introduced through the abnormal urethral opening into the perineum which was located in the midline raphe of the perineal region about 2 cm from the anal verge. This urethra joined with the penile urethra distal to the external sphincter. Prostatic part of the common urethral channel was normal containing verrumontanum and a normal bladder neck. Bangladesh Journal of Urology, Vol. 19, No. 1, Jan 2016 p.53-56

1989 ◽  
Vol 67 (4) ◽  
pp. 1053-1060 ◽  
Author(s):  
Bernard Lebeau ◽  
Gérard Pageau

Primary and secondary sexual characteristics in northern pike, Esox lucius Linneaus, and muskellunge, Esox masquinongy Mitchill, differ markedly. Differences in the internal urogenital anatomy include the structure of the genital ducts and the angle they form with the ventral surface. Externally, the genital and urinary ducts open directly to the environment in northern pike, whereas in muskellunge they open into a muscular papilla and then through a urogenital pore. Differences in sexual dimorphism between the two closely related esocids constitute a structural basis to explain the known differences in spawning behaviour and reproductive success. Differences in secondary sexual characteristics explain why the methods developed by Demchenko or Casselman to externally sex pike are inapplicable to muskellunge. To develop and test two external sexing techniques for muskellunge, specimens were collected from two regions in Canada from 1980 to 1987. Examination of reproductive products obtained from stripping provided an accurate indicator of sex and sexual maturity, but demonstrated serious limitations. A simple method to sex muskellunge by examination of the external urogenital morphology is described for juveniles and adults. Dissection of fish revealed that with the urogenital examination technique we sexed juveniles with a success rate of 92–98%, and adults with 100% accuracy.


2015 ◽  
Vol 218 (7) ◽  
pp. 603-615 ◽  
Author(s):  
Jia-Woei Hou ◽  
Ching-Ling Lin ◽  
Yen-An Tsai ◽  
Chia-Huang Chang ◽  
Kai-Wei Liao ◽  
...  

2021 ◽  
pp. 113-118
Author(s):  
V. A. Melnik

Objective: to assess the constitutional features of the age dynamics of secondary sexual characteristics and functional parameters of city schoolchildren in the age range of 7–17 years.Materials and methods. The objects of the study were schoolchildren aged 7–17. The stages of the expression of secondary sexual characteristics were determined visually according to the scheme by J.M. Tanner. The functional parameters of the examined schoolchildren were assessed using the methods of dynamometry, spirometry; blood pressure and heart rates, as well as the time of simple sensorimotor reactions were measured.Results. As a result of the performed studies, it has been found that representatives of the hypersomnic types are ahead of other types to reveal the first stages of secondary sexual characteristics. Schoolchildren with the leptosomic morphotypes due to higher rates of their development have reached the definitive stages by the same age as those with the hypersomnic variants. The intensity of age-related changes in functional parameters (strength qualities, circulatory and respiratory systems, nervous system) in the studied objects depends on their body type.Conclusion. The stage and rate of puberty, as well as the development of functional parameters in city schoolchildren depends on their somatotype.


2019 ◽  
Vol 7 (1) ◽  
pp. 6
Author(s):  
Shu-Heng Huang ◽  
Daw-Yang Hwang ◽  
Shang-Jyh Hwang

17α-hydroxylase deficiency (17OHD) is a rare disorder of secondary hypertension caused by congenital adrenal hyperplasia. In addition, co-occurrence of an adrenal tumor with 17OHD is extremely rare and easily misdiagnosed. A 33-year-old female with sicca syndrome, persistent hypertension, hypokalemia, and a left adrenal tumor was referred for confirmation of primary aldosteronism. However, the absence of secondary sexual characteristics, persistent growth beyond puberty, and laboratory data of low plasma renin activity, high aldosterone, low cortisol, low sex hormones, elevated adrenocorticotropic hormone, elevated luteinizing hormone, elevated follicle-stimulating hormone, and most importantly, decreased 17-hydroxypregnenolone, supported a diagnosis of 17OHD. We sequenced the CYP17A1 gene of the patient and her parents, which demonstrated genetic defects (D487-S,488-F489 deletion and Y329K418X). 17OHD was diagnosed. The left adrenal tumor was assessed, and a non-functional adrenal incidentaloma was confirmed; NP-59 adrenal cortical scintigraphy and adrenal venous sampling showed no functional activity and non-lateralization. Hormone replacements with estrogen, spironolactone, and prednisolone were given. The patient became more feminized and confident, and her hypertension was controlled. Early diagnosis and treatment of 17OHD not only can prevent delay development of secondary sexual characteristics but also help the patient maintain mental health and improve their quality of life. In addition, the concomitant presence of a left adrenal tumor makes misdiagnosis of a functional adenoma more likely, possibly causing unnecessary surgery and delay inappropriate treatment.


Author(s):  
Nidhi Jain ◽  
Jyotsna Harlalka Kamra

Primary amenorrhea is defined as failure to achieve menarche till age of 14 years in absence of normal secondary sexual characters or till 16 years irrespective of secondary sexual characters. The most common cause of primary amenorrhea is gonadal pathology followed by Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome). MRKH syndrome is a rare congenital disorder characterised by uterine and vaginal aplasia. It occurs due to failure of development of Müllerian duct. Its incidence is 1 per 4500 female births. Mostly girls present with primary amenorrhea. It is characterised by presence of normal secondary sexual characteristics, normal 46 XX genotype, normal ovarian function in most of the cases and absent or underdeveloped uterus and upper part (2/3) of vagina. It is of two types: type A is isolated type while type B is associated with other renal/skeletal/cardiac anomalies. Treatment includes psychological counselling and vaginoplasty. Vaginoplasty can be done by various non-surgical and surgical techniques. The authors hereby review the literature of MRKH syndrome regarding its embryology, etiopathogenesis, approach to work up and management.


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