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Complete hindgut duplication is a rare & intriguing entity, often coupled with genitourinary abnormalities và neural tube defects. The diagnosis demands a thorough clinical exam & radiological workup. Timely recognition và expeditious treatment of these patients can lead to a better unique of life. We present a case of a 10-month-old female with complete hindgut duplication và associated genitourinary duplication treated with surgical intervention.
Gastrointestinal (GI) duplications are a rare occurrence & may involve sầu any part of the alimentary tract. The diverse clinical presentations depend on the kích cỡ, site, và type of duplication. Complete hindgut duplication, an extremely rare condition, often presents with associated genitourinary duplications.
We present a case of total duplication of the hindgut. The patient also had a duplication of the genitourinary system. A đánh giá of the literature revealed only a handful of patients with the same condition. One case reported by Schatz had a complete duplication of bladder, urethra, vagimãng cầu, uterus, and anus <1>. The management of duplications varies greatly according to the anomalous anatomy. If surgical management is not undertaken, a possibility of neoplastic change should not be ruled out <2>. What makes our case more quality is that our patient had an associated situs inversus, which khổng lồ the best of our knowledge, has not been reported with any case of hindgut and genitourinary duplication. The purpose of our study is to lớn identify this rare set of associated conditions early because prompt surgical treatment has shown a favorable prognosis and better chất lượng of life for patients. Informed consent statement was obtained for this study.
A 10-month-old female born at term was brought to the outpatient department by her mother with a complaint of fecal discharge from the vagina since day twenty of birth và constipation with mild abdominal distention for the last two months. The mother had noted fecal discharge from the normal anal opening as well as the vagimãng cầu. Past medical, surgical và birth history were unremarkable. On examination, the patient was lying comfortably and was responsive sầu lớn her surroundings. Her vital signs were stable. Abdominal examination revealed soft abdomen with mild distention. There was no tenderness or any palpable mass & no signs of ascites. Bowel sounds were audible. Digital rectal examination (DRE) showed normal anal opening. On rectal exam, a soft bulge was felt on the anterior wall of the rectum with an impression of an extrinsic growth. There was a fistulous opening in the vestibule of the vagina from which fecal matter was exuding. Rest of the systemic examination revealed no abnormalities.
Routine laboratory investigations were normal. Contrast enema revealed duplication of the anal canal, rectum, sigmoid colon & situs inversus with liver on the left as shown in Figure Figure11.
Contrast enema abdominal radiograph showing duplicated sigmoid colon (small arrow), liver on the left side (large arrow)
On the basis of history, physical examination and investigations, a diagnosis of hindgut duplication were made and examination under anesthesia (EUA) and exploratory laparotomy (EL) was planned. During EUA, six perineal openings (duplicated vagina, urethra and anus) were revealed as shown in Figure Figure22.
Examination under anesthesia showing two urethral openings (small arrows at the top), two vaginal openings (large arrows), ectopic anal opening (star), and normal anal opening at the bottom (arrow below)
On contrast studies two urinary bladders and two vaginas were visible. EL revealed duplication of hindgut from anus till cecum/appendix as seen in Figure Figure33.
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Exploratory laparotomy showing duplicated cecum (large arrows) và appendix (small arrows)
Distally, the ectopic anus was excised & proximally the duplicated gut was internally drained into the normal rectum. The septum between the two rectums was divided with linear cutter stapling device lớn create one single sigmoid & rectum. The septum between the two bladders was divided and converted khổng lồ a single lumen viscera. The patient had an uneventful postoperative sầu period và was discharged on the fifth postoperative sầu day with a follow-up plan of care.
The repair of the double urethra was postponed till the age of three years where the urine continence status of the patient will determine further management. The management of double vagimãng cầu was postponed till puberty after assessing the menstrual & fertility status of the patient.
Gastrointestinal (GI) duplications are a very uncommon, but complex phối of embryological disorders can involve sầu any part of the alimentary canal from the tongue to lớn the anus. Duplications of any kind cốt truyện a few comtháng characteristics. They are hollow, lined with gastrointestinal tract epithelium & have sầu a smooth muscle wall.
GI duplications were initially believed to be more comtháng in males as compared khổng lồ females but it was later confirmed by a study that there is an equal male lớn female ratio <1>. Colonic duplications trương mục for 15% <3> và rectal duplications for 5% <4>. The exact pathogenesis of duplications is unknown và multiple theories have sầu been put forward. According to lớn the most credible theory, as the rapidly growing endothelial cells occlude the intestinal lumen, vacuoles size inside the cell masses due to the growth of the intestine. These vacuoles fuse to create a single lumen intestine. If one of these vacuoles pinches off, they can create a secondary lumen, which may entirely be separated from the main lumen but grows in proportion lớn the main lumen <5>.
Colonic duplications can be cystic or tubular involving a limited portion of the colon or be extensive sầu. They are usually divided inkhổng lồ two types, type I và II. Type I usually has partial involvement of the colon or rectum while type II has a wider spectrum as in addition to lớn colon & rectum, there can also be associated congenital anomalies including duplication of the lower genitourinary tract, double appendices, situs inversus & neural tube defects <5>.
The presentation of this condition might vary greatly from patient to patient depending upon the degree of và site of duplication. In a colonic or anal duplication, the patients are usually asymptomatic. If symptomatic, a colonic duplication can cause symptoms related lớn an obstruction such as abdominal pain, distention, và constipation. There can be concurrent fistulas in tubular colonic duplications with other surrounding structures or hollow viscera such as vagimãng cầu, vulva, perineum và urinary tract. Without a fistula, there can be a distention of the duplicated gut distally. In our patient, there was a presence of a recto-vaginal fistula, which presented as fecal discharge from the vagina initially. The patient also had mild progressive abdominal distention afterward.
Urogenital duplication can present as a separate entity or in association with hindgut duplication. The constellation of these associated abnormalities can be explained by "partial twinning" of the caudal kết thúc of the embryo or abnormal development of urorectal septum <6>. Duplication can either be complete or partial và can occur in any part of the urogenital tract including bladder, urethra, and vagina. Although duplication may occur in the sagittal or coronal plane, it is more commonly seen in the former. The anatomy of urogenital duplications determines if a patient presents earlier or later in life. With earlier presentations, urinary tract infections, obstructions, and voiding difficulties are usually seen. With later presentations, the patients remain largely asymptomatic & occasionally present with infertility <7-8>.
Various diagnostic modalities are available for the diagnosis of GI duplications. Ultrasonography and contrast studies are most widely used. Computed tomography (CT) and magnetic resonance imaging (MRI), although less often used, are helpful in localizing và diagnosing complex duplications <9>. Treatment of duplications largely depends on whether or not a patient is symptomatic. Generally, a conservative approach is followed if no symptoms are seen. If surgery is considered, the primary objective is khổng lồ treat the patient"s symptoms rather than correct the anatomical abnormality. The choice of surgery depends upon two important factors. Firstly, care should be taken not khổng lồ affect the blood supply of the normal bowel as the duplicated segment might giới thiệu its blood supply with the normal bowel segment. Secondly, the internal drainage of the duplicated segment containing ectopic gastric mucosa can lead to gastrointestinal bleeding. Cystic duplications are easier khổng lồ excise because their site of involvement is limited while tubular duplications need as extensive sầu resection <10>.
Although extremely rare, hindgut duplications, if found, should be investigated and a clinical-radiological correlation should be made to accurately delineate the site & extent of the disease. Also, one must be vigilant, as these duplications are often associated with other systemic anomalies. Management of the duplication depends upon the extent and symptomatology of the disease, as larger and symptomatic duplications require more aggressive surgical management.
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