Hindgut Duplication Research Articles

Variations in Epithelial Lining of Duplication Cysts Cannot be Explained by Known Theories.

Objectives. Duplication cysts are found in any part of the gastrointestinal tract from the oropharynx to anus. Although duplication cysts usually have similar epithelium with the adjacent organ, respiratory epithelium in the enteric duplication cysts is rarely reported. This study was performed to evaluate the variations in the epithelial lining of duplication cysts and its clinical implications. Methods. Patients diagnosed with duplication cysts between 2012 and 2022 were retrospectively reviewed to assess their histopathological results, clinical aspects, treatment options, and demographic characteristics. Results. Twenty-five patients were included. The mean age was 4 years, male-to-female ratio was 15:10. The localizations of duplication cysts were ileum (n = 12), duodenum(n = 4), stomach(n = 3), jejunum(n = 2), colon(n = 2), thoracoabdominal(n = 2), and rectum(n = 1). Most common presentation was abdominal pain (36%). Intestinal (48%) and gastric (40%) epithelia were the most common finding. Four patients (16%) had respiratory epithelium: two in foregut duplication cysts, one each in ileal, and rectal duplications. Twenty-four percentage of patients had associated anomalies. Surgical interventions were performed on 22 patients. Conclusion. Duplication cysts show a wide range of epithelial linings. Respiratory epithelium can be found in not only foregut duplication cysts but also midgut and hindgut duplication cysts. Although the presence of respiratory epithelium did not have any impact on clinical findings, none of the previous theories explain the presence of respiratory epithelium different from foregut duplications.

Caudal duplication syndrome: Complete duplication of the hindgut and genitourinary system

Caudal duplication syndrome is a very rare anomaly and poses a major challenge for surgeons. The term caudal duplication syndrome is used to describe the association between hindgut duplication, duplication of the genitourinary, and malformations of the spinal cord and vertebrae. A 5-year-old girl is reported with complete duplication of the entire large bowel, cecum, appendix, about 2 cm of the terminal ileum, bladder, urethra, vagina, uterus, and vertebral body deformities. After an extensive evaluation phase, a laparotomy was performed and the duplicated colon was excised completely without removing the adjacent native colon.

Covered bladder exstrophy with heterotopic hind gut duplication cyst: A case report with review of literature

There are many different variants of classical bladder exstrophy (BE) and covered BE is one of these rare variants which has an association with heterotropic hind gut duplication cyst. We report a case of a 20 months old girl with covered BE, who had a 5 cm cystic mass attached anteriorly to the bladder, which turned out to be a hind gut duplication cyst on histopathology examination. So a possibility of a heterotopic hind gut duplication cyst should be kept in mind if a cystic mass is found anterior to a covered BE.

Caudal Duplication Syndrome Systematic Review-A Need for Better Multidisciplinary Surgical Approach and Follow-Up.

Background and Objectives: Caudal duplication syndrome is a rare association of anatomical anomalies describing duplication of the hindgut, spine, and uro-genital structures, leading to varied clinical presentations. The current literature focuses on case reports which describe the embryological etiology and anatomical spectrum of the condition giving little attention to the surgical preparation, the need for a well-structured follow-up program, or the transition into adult healthcare of these complex patients. No reviews have been published regarding this complex pathology. Materials and Methods: A review of caudal duplication syndrome cases was done to assess the range of the clinical malformations, timing, and types of surgical interventions. Inconsistencies in multidisciplinary care, follow-up, and risk events were described. Results: Hindgut duplication always involved the anorectal region. Anorectal malformations were evenly distributed as unilateral and bilateral. Colon duplication extended from the anal region to the transverse colon or ascending colon in most of the cases and less to terminal. In females, genital duplication was present in all cases. The follow-up period varied between 3 months and 12 years. In all adult females, the motive of presentation was related to pregnancy (complications after successful delivery, fertility evaluation) or late complications (fecalith obstruction of the end-to-side colon anastomosis, repeated UTIs with renal scarring). Conclusions: Complex malformations affecting multiple caudal organs may have a strong impact in many aspects of the long-term quality of life; therefore, patients with caudal duplication syndrome need increased awareness and joined multidisciplinary treatment.

Incomplete bladder duplication with total Hindgut duplication: a rare case

Incomplete bladder duplication with total Hindgut duplication: a rare case

Sacrococcygeal teratoma associated with hindgut duplication: A case report & review of literature

The index case report describes a previously unreported coexistence of sacrococcygeal teratoma and a tubular duplication of the rectum. The child presented with a large sacrococcygeal mass. He was treated with pre-operative chemotherapy to reduce the bulk of the tumor and subsequently underwent surgical resection. Exploration surprisingly revealed an additional long tubular duplication of the rectum, which did not share any lumen with the parent gut. The duplication cyst and the residual tumor were excised. The histopathology report confirmed residual mature teratoma and the duplication cyst. Relevant literature is reviewed.

A Rare Presentation of Caudal Duplication Syndrome in an Adult with No Functional Impairment

Caudal duplication syndrome is a rare congenital association between malformations and duplications of the gastrointestinal and genitourinary systems and neural tube defects. Less than 100 cases have been reported in literature worldwide, majority being diagnosed at birth. To date only 2 patients have been reported to have presented in adulthood. We hereby report a case of caudal duplication with complete duplication of urethra, bladder, vagina, uterus, entire hindgut and midgut but with no functional abnormalities. Her only complaint being narrow double vaginal vaults with anticipated difficulty in sexual function. As normal pregnancy has been reported in uterus didelphys, a simple vaginal septal resection and posterior vaginal wall lengthening with incorporation of a ‘Z-plasty’ was done along with vulvaplasty to exclude the abnormal anal opening from the fourchette.

Tubular Hindgut Duplication with Rectovaginal Fistula, Dysplastic Kidney and Ectopic Ureter: case report

Tubular Hindgut Duplication with Rectovaginal Fistula, Dysplastic Kidney and Ectopic Ureter: case report

Anal canal duplication associated with presacral cyst

Anal canal duplications are rare congenital malformations, with fewer than 50 reported cases in the literature. Anal canal duplications are noncommunicating second anal orifices located posterior to the true anus without other associated hindgut duplications. Typically, these are asymptomatic, tubular malformations that present in females before the age of 6 years. Here, we report on a 16-year-old girl with a symptomatic anal canal duplication associated with a presacral cystic component. This is an unusual presentation of an already rare entity. An overview of the clinical presentation, radiologic workup, surgical treatment, and histologic features of anal canal duplications is provided.

V-004 Hindgut Duplicationの直腸膣瘻に対し,仙骨アプローチで手術した1歳女児の1例(第44回日本小児外科学会学術集会)

V-004 Hindgut Duplicationの直腸膣瘻に対し,仙骨アプローチで手術した1歳女児の1例(第44回日本小児外科学会学術集会)

Congenital double anus with total colon duplication: a case report

Cases of congenital double anus are very rare. The number of cases of hindgut duplication of all kinds is slightly higher, and some series include patients with genitourinary and genitalia duplications but with a single anus. However, double termination with tubular duplication is rarely heard of. We report on the case of a patient with 2 well-formed ani, total colon duplication, and 2 vaginas.

Complete unilateral leg duplication with ipsilateral renal agenesis

A case of a 5-month-old infant with complete duplication of the right leg and ipsilateral renal agenesis is presented. There was also a duplicated scrotum, posterior urethral valves with hydronephrosis, abnormal pelvic bones, and a ventricular septal defect. Complete caudal duplication is exceedingly rare. Associated defects are common. Several aetiological theories have been proposed. Among them, an underlying polytopic (acro-renal) developmental field defect has been suggested. It best explains the specific findings in our patient. However, the aetiology of caudal duplication syndromes may be multifactorial. In the light of twinning theories, associated duplication of hindgut derivates should be suspected in similar cases. Further research is needed. The motor-skeletal functional outcome after leg duplication surgery is mostly favourable. A case of complete unilateral leg duplication with ipsilateral renal agenesis is presented. It appears to support the theory of a polytopic developmental field defect.

Triplication of colon with diphallus and complete duplication of bladder and urethra

Gastrointestinal duplications are a common type of congenital anomaly usually restricted to one part of the gut. Triplication of colon is a rare variation of duplication of the hindgut. Hindgut duplications are commonly associated with anomalies of other derivatives of the cloaca, namely, the lower genitourinary system. Here, we describe a child with triplication of colon with associated duplication of bladder and urethra and diphallus. The child described here is the only reported survivor with triplication.

Anorectal malformation with tubular hindgut duplication

The association of hindgut duplication and anorectal malformation is rare. Published classifications of this association are confusing in respect of terminology. We report a case of blind-ending, Y-shaped tubular duplication of the distal hindgut, associated with an anorectal malformation (rectourethral fistula) affecting the colon proper. Surgical options at time of presentation and of reconstructive surgery are discussed. A review and suggested modification of the classifications is presented.

Duplications of the alimentary tract.

A review of 53 patients with 60 duplications of the alimentary tract treated at the Royal Hospital for Sick Children, Glasgow, from 1947 to 1978 has been made. This consisted of 19 foregut, 32 midgut and 9 hindgut duplications. The experience with these patients has been recorded and discussed with reference to the literature.

Duplications of the alimentary tract.

A review of 53 patients with 60 duplications of the alimentary tract treated at the Royal Hospital for Sick Children, Glasgow, from 1947 to 1978 has been made. This consisted of 19 foregut, 32 midgut and 9 hindgut duplications. The experience with these patients has been recorded and discussed with reference to the literature.

Prenatal diagnosis of cystic bladder distension secondary to obstructive uropathy

We report the perinatal findings of a huge midline posterior cystic bladder distension secondary to lower urinary tract obstruction and prune-belly syndrome in a male fetus. A 40-year-old woman, gravida 3, para 0, was referred at 21 gestation weeks with sonographic findings of anhydramnios and a fetus with a 9.5 x 6.0 cm intra-abdominal cystic mass containing two chambers. The in utero ultrasound-guided fetal bladder drainage using a single needle aspiration and the ultrasound follow-ups of fetal bladder filling provided a diagnostic aid. This method helped to show the position of the bladder and the cystic bladder mass as well as the status of communication in response to decompression or filling of the fetal bladder. Cytogenetic analysis revealed a 46,XY karyotype. Autopsy showed agenesis of the posterior urethra, prominent megacystis, a cystically distended mass arising from the lower posterior bladder, hydronephrosis, megaureters, and anorectal agenesis with an intestinal blind end adherent to the posterior wall of the uterus. There were no urogenital duplication, hindgut duplication, or urachal abnormalities. The contracted bladder had a full-thickness muscular wall with a trigone and two ureteral orifices while the cystically distended bladder did not have any opening and was lined by a very thin wall. Histology of the cystic bladder wall demonstrated typical urothelium, lamina propria and muscularis propria. The pathogenesis and differential diagnosis of cystic bladder distension are discussed.

Colonic duplication: An unusual cause of enterovesical fistula

Intestinal duplications are rare, occurring in 1 in 4000 births. Even rarer are tubular hindgut duplications, with less than 75 cases reported in the literature to date. We report an uncommon case of an enterovesical fistula caused by a duplication of the sigmoid colon. This is the first reported case of a tubular colonic duplication presenting as a colovesical fistula in an adult. We discuss the presentation and shortcomings in the diagnosis and treatment of enterovesical fistulas and colonic duplications.

A Cloacal Exstrophy Variant Associated with Hindgut Duplication

A Cloacal Exstrophy Variant Associated with Hindgut Duplication

Heterotopic hindgut duplication: A cloacal remnant associated with exstrophy of the bladder

Lower hindgut duplications, although very rare, usually present as a cystic mass located posterior to the rectum and confined to the pelvis. Hindgut duplications are often associated with genitourinary or spinal anomalies. We describe a neonate with a previously unreported heterotopic colonic duplication presenting as an exophytic perineal mass associated with classic bladder exstrophy. The embryonic significance of these anomalies is discussed.