Complete diphallia associated with unusual multiple congenital anomalies: case report and review of literatures

Background Diphallus (duplication of phallus) is rarely encountered in surgical practice with only 100 cases reported in literature. Some cases may be isolated but mostly associated with other anomalies, without clear data about its etiology. Case presentation We reported a 1-day-old newborn baby who was presented with complete duplication of the phallus, one of them being hypospadic associated with a high type imperforate anus, omphalocele, congenital pouch colon, sacral meningocele, and other congenital anomalies not reported before in such combinations. Multiple stages surgical corrective procedures were performed over a period of 4 years with 4–6 months interval between each stage, starting with the management of omphalocele and colostomy, ended by excision of the abnormal phallus with abdominoplasty and closure of colostomy. The outcome was evaluated, and literatures were reviewed in relation to types, presentations, and options for surgical correction with optimal outcomes. Conclusion A combination of diphallia and other abnormalities in our patient are not reported previously in such manner and were very difficult to be corrected. Only expert pediatric surgeons should treat such conditions as every case is unique in nature. In all conditions, the abnormal phallus should be excised, and the final aim is to achieve a continent child with cosmetically acceptable genitalia.

Congenital Pouch Colon in a Neonate

Congenital Pouch Colon (CPC) is a rare anorectal malformation (ARM) in which a part of or the entire colon is replaced by pouch-like dilatation. Males are more likely to be diagnosed with the condition compared to females. The highest incidence of the disease is in South Asia, with a significant number of cases reported from India. Early diagnosis can be made when there are hypoechogenic lesions on antenatal ultrasound scans. We report a case of a neonate with routine antenatal scans who presented with a distended abdomen and inability to pass feces. The diagnosis was made in the early neonatal period, followed by surgical management. doi: https://doi.org/10.12669/pjms.38.ICON-2022.5771 How to cite this:Niaz S, Naz S, Raziq RA. Congenital Pouch Colon in a Neonate. Pak J Med Sci. 2022;38(2):426-429. doi: https://doi.org/10.12669/pjms.38.ICON-2022.5771 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

RARE COLORECTAL MALFORMATIONS IN NEWBORNS. LITERATURE REVIEW AND OWN OBSERVATIONS

The article presents literature data andown observations of some rare congenital malformationsof the colorectal area in newborns that require surgicaltreatment. These are defects such as congenital pouch colonand congenital segmental dilatation of the colon. Thereare isolated cases of birth of children with these defectsin European countries and North America. They are morecommon in Asian countries, especially in India. Diagnosisand surgical correction of such congenital malformationscause difficulties associated with insufficient awarenessof pediatric surgeons, neonatologists, pediatricians aboutthis pathology. Data is presented on clinical manifestations,antenatal and postnatal diagnosis and treatment tactics, aswell as the histopathological structure of the affected colonin these defects.The analysis of literature data and the presented clinicalobservations of congenital pouch colon and congenitalsegmental dilatation of the colon in newborns indicate thepossibility of their antenatal and preoperative diagnosis.It is concluded that children with anorectal malformationsand Hirschsprung's disease require a thorough differentialdiagnosis with congenital pouch colon and segmentaldilatation of the colon, as the tactics of their surgicalcorrection significantly differ from the treatment tacticsof common anorectal malformations and Hirschsprung'sdisease surgical management.

A case of prenatally diagnosed prune belly syndrome variant and congenital pouch colon in the United States: A case report

Background: Prune belly syndrome (PBS) and congenital pouch colon (CPC) are rare congenital syndromes with a low incidence in the United States (U.S.) with most CPC cases being from India. In this case report, we describe, to the best of our knowledge, the first PBS variant and CPC patient in the U.S. Case Presentation: A 30-year-old G2P0010 woman was referred to a tertiary center after an 18-week ultrasound showed a fetal abdominal mass. A prenatal MRI showed a dilated loop of bowel containing a mixture of urine and meconium, oligohydramnios, and a protuberant abdominal wall. Born at 37 weeks, the child’s physical exam was notable for a distended abdomen with thin abdominal musculature, non-palpable bilateral testes, no anal opening, and flat buttocks. Intra-operatively, a dilated cecum/ascending colon was noted with an abrupt change in caliber at the transverse colon, bilateral enlarged ureters, a left testis at the internal ring and no visualized right testis. A colostomy and mucous fistula were created 5 cm from the sigmoid pouch. Conclusion: While most reported cases of CPC undergo single stage repair (one operation) at 1 day of life, our patient underwent the first procedure of a staged repair at 16 hours of life given his clinical instability at the time as well as his unknown urological anatomy in the setting of urinary obstruction. This case demonstrates the importance of fetal imaging, multidisciplinary approach at a tertiary care center, and reinforces a staged repair when necessary.