Absence Of External Genitalia Research Articles

Surgical Management of Sirenomelia: A Case Study.

Sirenomelia is a rare congenital condition characterized by fusion of the lower limbs. Patients with sirenomelia generally do not survive long after birth because the condition is associated with multisystem organ dysfunction due to developmental anomalies. Considering the low incidence and few cases surviving the neonatal period, there is minimal understanding regarding the surgical management of sirenomelia. We present a unique case of an infant born with type 1 sirenomelia, absence of external genitalia, presence of a cloaca, absence of the bladder, and presence of an imperforate and vestigial anus, who not only survived the birth process, but, at the age of 11 months, was determined to be a candidate for surgical separation of the lower extremities. This case was approached much like a dorsal rectangular flap syndactyly release. Large Z-plasty flaps were designed and raised, and the soft tissue within the skin bridge was meticulously dissected to preserve anatomy and to provide adequate skin flaps without perineal skin grafting. A quadrangular flap was designed to reconstruct the perineum and produce a neo-vulva using de-epithelialization. Successful lower extremity separation was achieved. There were no major postoperative complications. The patient progressed with lower extremity function, and eventually achieved independent ambulation. Management of sirenomelia is incredibly challenging, and data to guide surgical management are limited. This report details our approach to a successful lower extremity separation, repair, and neo-vulvar reconstruction in a case of type I sirenomelia.

Embryological analysis of a case of exstrophy of the cloaca

A case is reported here of a stillborn fetus of about 21 weeks of gestation that was collected from the Department of Obstetrics and Gynecology, Regional Institute of Medical Sciences (RIMS), Imphal, Manipur, India. The weight of the fetus was around 350 g. Crown rump (CR) length was not taken because of spine deformity and, therefore, the foot length, which measured 3.3 cm, was used to determine the age of the fetus. External examination of the fetus revealed an enormous defect in the anterior abdomen wall (omphalocele). The umbilical cord was attached on the right side of omphalocele. Incision of the membranous sac revealed liver, coils of intestine, and a muscular sac herniating through the defect in the abdominal wall. Further exploration of the abdomen showed continuity of the large intestine and ureters to the muscular sac. The sac is the cloaca. Exstrophy of the cloaca is a rare developmental anomaly involving the urinary bladder and the distal part of the digestive tract. This case report discusses the embryological background of the development of exstrophy of the cloaca and is reported because of the association of complete absence of external genitalia.

Gastroschisis with Multiple Skeletal Deformities, Imperforate Anus and Absent Genitalia : A Rare Presentation

Gastroschisis is a rare type of congenital abdominal wall defect involving full thickness of anterior abdominal wall. Out of different varieties, gastroschisis is one of the severe forms with an incidence of about 1 in 10,000 live births. The deform- ity is more commonly seen in young mothers less than 20 years. They are usually small for gestational age and have a high mortality rate. Only 60% survive till the end of first year. Gastroschisis with intact bowel is known as simple and those with intestinal atresia, perforation and/or necrosis are called complex gastroschisis. Usually it is not associated with any other congenital malformations. We present a rare case of gastroschisis associated with other anomalies like limb defects, kyphosis, talipes equinovarus, imperforate anus, absence of external genitalia and nipples. This congenitally abnormal fetus was delivered by a 23 year old primy at 24 weeks of gestation and was diagnosed by ultrasound.

Sirenomelia associada a defeitos congênitos raros: relato de três casos

Sirenomelia, an extremely rare congenital defect, is defined as a limb abnormality in which the normally paired lower limbs are replaced by a single midline limb. It is commonly associated with varied genitourinary anomalies. We report three cases of sirenomelia including x-ray documentation of the lower limb. Other associated aspects, whose diagnoses were established exclusively through autopsy, included bilateral renal, ureteral and bladder agenesis, rectal atresia, imperforate anus, intra-abdominal testis, absence of external genitalia and other rare congenital abnormalities.

Raccoon Populations: Does Human Disturbance Increase Mortality?

Human disturbance is thought to be the major cause of mortality in raccoon (Procyon lotor) populations in North America. To test that hypothesis, characteristics of raccoon populations associated with low human disturbance (no trapping, low vehicular traffic, etc.) and high human disturbance (trapping, moderate vehicular traffic, etc.) were compared in the Manhattan, Kansas area in fall 1986. Estimated annual mortality in the raccoon population exposed to little human disturbance was 26.5%, significantly lower than the 52.5% in the population inhabiting an area of high human disturbance. Counts of placental scars reflected greater reproductive activity in the raccoon population in the highly disturbed area. The higher reproductive activity of raccoons inhabiting the area of high human disturbance may offset the higher mortality experienced by that population. The raccoon (Procyon lotor) is an adaptable mammal with a widespread range in North America (Sanderson, 1987). Stains (1956) presented data that characterized general parameters of raccoon populations in Kansas. Sanderson (1987) concluded from several studies that human activities are the primary causes of mortality in raccoon populations. To test Sanderson's conclusion, the characteristics of a raccoon population inhabiting an area with a large amount of human disturbance were compared to those of a raccoon population exposed to little human disturbance. Specifically, it was hypothesized that the raccoon population in the area with greater human disturbance would have higher mortality than the population experiencing little human disturbance. Herein, are the results of that comparison. MATERIALS AND METHODS Body weight, sex, age, and reproductive data were obtained from raccoons collected from two areas in northeastern Kansas during November and This content downloaded from 157.55.39.173 on Thu, 19 May 2016 06:18:36 UTC All use subject to http://about.jstor.org/terms VOLUME 93, NUMBERS 1-2 23 December 1986. One area was the Fort Riley Military Installation (minimal human activity, few residences, little vehicular traffic, no trapping, no farming, etc.), whereas the other was the rural Manhattan area (higher human activity, farm homes, moderate vehicular traffic, trapping, farming, etc.), 20 km to the northeast. The study areas were on the western edge of the Flint Hills of Kansas. Topography of the region is dominated by rolling prairie intersected with small drainages of intermittent streams. Elevation ranges from 400 to 500 m. Specific boundaries of the study areas are described in Barnes (1987), and vegetation and topography are detailed in Robel et al. (1970) and Klinger (1983). Techniques used to collect raccoons were identical on both study areas, i.e., water sets using steel coil-spring and long-spring leghold traps. Thirtysix raccoons were collected from the Fort Riley area and 92 raccoon carcasses were obtained from furharvesters trapping in the rural Manhattan area. The weight of each raccoon trapped on Fort Riley was determined prior to skinning; body weight data were not recorded for raccoons received from the cooperating furharvesters because the skinned carcasses had dried for various lengths of time before we obtained them. A preliminary determination of sex was made by presence or absence of external genitalia and confirmed by inspection of internal gonads. Age was determined by examining canine root foramina and counting cementum annuli of canine teeth (Grau et al., 1970). Reproductive history was determined by examining uteri of females and counting placental scars. Standard Chi-square, Student's t, F values, and LSD analyses were used to detect significant differences in the data sets at a 0.05 level of probability. Survival of raccoons in the two populations was estimated from trapping results following procedures of Robson and Chapman (1961) as detailed by Eberhardt (1969). For these calculations, we assumed both raccoon populations were static and our trapping efforts provided an unbiased representation of the age classes in each population. Because annual survival and mortality must add up to unity, one minus survival equals mortality. RESULTS AND DISCUSSION Weights of adult raccoons from Fort Riley (8.6 kg) were greater than juveniles (4.1 kg), and adult males (9.8 kg) were heavier than adult females (8.1 kg). Body weights of our adult raccoons were higher than reported in Illinois (Sanderson and Hubert, 1981). Body weight of juvenile male raccoons did not differ from that of juvenile females (Table 1). The proportion of male (39%) raccoons from Fort Riley was not significantly different from the proportion of males (47%) obtained from the rural Manhattan area. Most other studies report higher proportions of male raccoons than females (Sanderson, 1951; Johnson, 1970; Moore and Kennedy, 1985). This is thought to be a reflection of the higher vulnerability of males This content downloaded from 157.55.39.173 on Thu, 19 May 2016 06:18:36 UTC All use subject to http://about.jstor.org/terms 24 TRANSACTIONS OF THE KANSAS ACADEMY OF SCIENCE Table 1. Body weights (kg) of raccoons collected on the Fort Riley Military Installation during November and December, 1986. Category n Body weight (C _+ SE)'