The rare, complex syndrome includes a wide spectrum of malformations ranging from partial or isolated to complete<unk>  of<unk>  organs in GI, GU, and neural systems. The syndrome may cause functional impairments such as an imperforate anus and hernia which may lead to death due to shock and organ failure and require prompt surgical intervention, but most presented symptoms are not life-threatening and duplicated organs are in fact functional in many cases. For instance, patients with genital<unk>  are mostly expected to have normal menstruation, sexual intercourse, and even pregnancy, although their self-esteem and quality of life may be influenced. Since the clinical presentation of each patient and its complexity vary greatly, the management which usually includes surgery are carefully planned and individualised based on the extent of<unk>  and functionality of the involved organs. An extensive medical work-up is required primarily before prognosis to understand the anatomy of patients and to decide appropriate treatment. Imaging modalities such as echocardiography, conventional X-ray, magnetic resonance imaging (MRI), ultrasonography, barium enema, computed tomography (CT) scan, and voiding cystourethrography (VCU) can be used to examine anomalies in detail. Exploratory laparotomy can also be conducted when needed. In most cases, surgical approach is utilised to excise or fuse the duplicated organs; however, surgical intervention is not a compulsory procedure for patients that do not exhibit functional deterioration and symptoms. Based on the work-up results, a multidisciplinary team consisting of a (paediatric) surgeon, a urologist, and a neurosurgeon plans individualised, staged correction. If a prenatal or after birth diagnosis is made, medical paediatric and surgery care are organised soon after delivery. Adults with the syndrome, however, usually do not require surgical treatment unless accompanied by symptoms or psychological issues. Reconstructive surgeries are performed to resolve the issue of functional impairments such as obstruction of colon, anatomic anomalies that hinder movement or cause infertility, and to improve cosmetic appearances in the case of genital<unk> . Gastrointestinal tract Treatments for colonic<unk>  varies from conservative management for asymptomatic cases to excision of duplicated colon to avoid potential issues such as colon structure and obstruction. Resection is possible when each duplicated colon has a complete blood supply. If the duplicated colons share a wall, a septotomy can be performed to create a small hole to connect two colons. In cases where rectum is also duplicated, either the rectums should be converted into one reservoir through septotomy followed by anorectoplasty or the duplicated colon and rectum should be removed and colostomies should be constructed. For patients with poor prognosis for bowel control, a Malone procedure can be utilised during the colostomy. Alternatively, in cases of renal<unk> , the Mitrofanoff procedure is performed instead. Genitourinary tract<unk>  of genital tract that does not involve functional impairment does not require surgical intervention; however, plastic surgery can be carried out to improve patients’ self-esteem and social status. For<unk>  of female genital tract, the septum between duplicated organs such as vagina, cervix, and vulva are resected to combine two duplicated organs into one or one duplicated organ could be detached and excised. For male patients, one duplicated genitalia can be removed, and duplicated scrotum and testis can be either combined or excised. The external genitalia of both male and female can be reconstructed by midline apposition of tissues. For<unk>  of urinary tract, bladders can be combined to form a single larger bladder without altering the duplicated ureters. Spinal and lower limb tract In cases of spine<unk> , prophylactic surgery can be done to remove one duplicated spine, although it depends on the severity of<unk> . In cases of malformation of the neural cord such as myelomeningocele and tethered cord which accompanies severe symptoms, preserving neurological function is the utmost importance by closing myelomeningocele and detethering the neural cord. This is a highly complex procedure that requires extreme caution not to injure the rectum in which case can cause a cerebrospinal fluid infection. For<unk>  of lower limb which not only hinders patients’ gait but also is highly visible and may affect patients’ self-esteem, excision of the supernumerary, non-functional pair of limb is often carried out. Due to the spectrum of symptoms present within<unk> , there is no uniform surgical treatment in relieving symptoms. The type and severity of surgical intervention is often dependent upon the type and complexity of symptoms presented. Thus, the primary goal of surgical treatment for the syndrome is to relieve symptoms, not to restore normal anatomy, and hence, potentially life-threatening malformations are addressed first and often followed by other anatomic or aesthetic reconstructions in later stages. After the medical treatment, the patients are periodically monitored.
Caudal duplication
Isodisomy
Robertsonian translocation
Genomic imprinting
Bromism
Trisomy
Germline mosaicism
Uniparental disomy