Isolated bladder exstrophy with normal phallus and imperforated anus: "A case report".

This case report presents a rare occurrence of isolated bladder exstrophy with a normal phallus in a one-day-old male newborn. The patient also presented with imperforated anus, congenital heart disease, and a left ectopic kidney. Surgical interventions included cut-back anoplasty, bilateral ureteral reimplantation, and primary bladder closure in a single operation, resulting in a successful outcome without complications. Three months of follow-up showed satisfactory results.

Role of ultrasound scan in prenatal diagnosis of cloacal exstrophy.

Cloacal exstrophy is a rare and complex ventral abdominal wall defect. Prenatal diagnosis is possible with the use of detailed ultrasound scanning. Prenatal diagnosis of an anomalous fetus helps the parents and their families in making informed decisions. This write-up is to report a case of cloacal exstrophy diagnosed during the prenatal period using an ultrasound scan. The pregnant mother presented at 35 weeks of gestation underwent a detailed ultrasound scanning and note the findings; a large defect on the ventral surface of the anterior abdominal wall extending from the umbilicus to the inferior pelvis, with a large mass protruding through the defect, umbilical cord arising from the proximal part of the mass, with another tubular mass protruding from the inferior aspect of the larger mass giving an appearance of "elephant trunk," absent urinary bladder, absent anal dimple, and scrotum was seen inferior to the mass but failed to appreciate the phallus. Both kidneys were normal, and there was no spinal defect. In addition, pubic bones were widely separated, and there was splaying of the iliac bone. Based on the ultrasound scan findings, a prenatal diagnosis of cloacal exstrophy was made. The pregnant mother and their families were counseled about the anomalous fetus, and they decided to continue the pregnancy. At birth, the abnormalities present in the baby were consistent with the prenatal ultrasound scan findings. On postnatal day 5, the baby underwent a complete primary surgical repair of the defect which comprised of closure of the abdominal wall defect, repair of bladder defect, pelvic osteotomies, and creation of a colostomy. The abnormalities of cloacal exstrophy detected by the ultrasound scan during the prenatal diagnosis period were consistent with the abnormalities detected at birth. This concludes that an ultrasound scan is a reliable tool to diagnose cloacal exstrophy during the prenatal period.

Caudal regression syndrome: Postnatal radiological diagnosis with literature review of 83 cases.

Caudal regression syndrome (CRS) is a rare congenital disorder characterized by arrest of caudal spinal growth and associated with wide spectrum multisystemic anomalies. Herein, we presented a case of a newborn baby who did not pass meconium due to imperforated anus and was referred to the pediatric surgeon for urgent diverting loop colostomy. The conventional X-ray, abdominal ultrasound and abdominal pelvic magnetic resonance imaging (1.5 T) at 2-month-old age revealed right kidney agenesis, sacrococcygeal agenesis, vertebral bodies dysraphism and the spinal cord ends at D12-L1 with anterior and posterior bands of the terminating filaments. The diagnosis of CRS was confirmed. Through this case report, we hope to draw attention to this rare syndrome and the wide range of associated anomalies, also to consider this syndrome on the top of differential diagnosis list once the newborn has anorectal malformation mainly imperforated anus.

Intraoperative visualization of urethra using illuminating catheter in laparoscopy-assisted anorectoplasty for imperforated anus-A novel and safe technique for preventing urethral injury.

PURPOSE: In this study, we used a near-infrared ray catheter (NIRC) to visualize the urethral line. We herein report our intraoperative visualization technique of the urethra using an illuminating catheter in laparoscopy-assisted anorectoplasty (LAARP) for imperforated anus. PATIENT AND SURGICAL TECHNIQUE: A 3.0-kg term male neonate with anorectal malformation was delivered. An invertogram revealed the type as intermediate. Transverse colostomy was performed at the left upper abdomen. A recto-bulbar urethral fistula (RBUF) was diagnosed via distal colostogram and voiding cystourethrogram. LAARP was planned at 6 months of age. We performed the operation with four trocars. A 45° 5-mm scope was used to clearly view the deep pelvic space. Before starting rectal dissection, a 6-Fr pig-tail-type NIRC was inserted through the external opening of the urethra to visualize the urethra during the laparoscopic procedure. The catheter tip was placed in the bladder, and excretion of urine was maintained through the NIRC during the procedures. While dissecting the deep pelvic space between the posterior wall of the urethra and anterior wall of the rectum, the exact line of the urethra was clearly confirmed by overlay images of the NIRC. The RBUF was dissected safely using this innovative image-guided technique. Anoplasty was performed between the rectal stump and perineal skin. The postoperative course was uneventful. Oral intake was started on postoperative day 1. Postoperative dynamic urography showed no complications. CONCLUSION: An NIRC is useful for detecting the urethra during LAARP.

Müllerian duct anomalies with term pregnancy: a case report.

BACKGROUND: Müllerian duct anomaly is a rare condition. Many cases remain unidentified, especially if asymptomatic. Thus, it is difficult to determine the actual incidence. Müllerian duct anomaly is associated with a wide range of gynecological and obstetric complications, namely infertility, endometriosis, urinary tract anomalies, and preterm delivery. Furthermore, congenital anomalies in pregnant mothers have a high risk of being genetically transmitted to their offspring. CASE PRESENTATION: We report a case of a patient with unsuspected müllerian duct anomaly in a term pregnancy. A 33-year-old Malay woman with previously uninvestigated involuntary primary infertility for 4 years presented with acute right pyelonephritis in labor at 38 weeks of gestation. She has had multiple congenital anomalies since birth and had undergone numerous surgeries during childhood. Her range of congenital defects included hydrocephalus, for which she was put on a ventriculoperitoneal shunt; imperforated anus; and tracheoesophageal fistula with a history of multiples surgeries. In addition, she had a shorter right lower limb length with limping gait. Her physical examination revealed a transverse scar at the right hypochondrium and multiple scars at the posterior thoracic region, levels T10-T12. Abdominal palpation revealed a term size uterus that was deviated to the left, with a singleton fetus in a nonengaged cephalic presentation. The cervical os was closed, but stricture bands were present on the vagina from the upper third until the fornices posteriorly. She also had multiple rectal prolapses and strictures over the rectum due to previous anorectoplasty. An emergency cesarean delivery was performed in view of the history of anorectoplasty, vaginal stricture, and infertility. Intraoperative findings showed a left unicornuate uterus with a communicating right rudimentary horn. CONCLUSION: Most cases of müllerian duct anomaly remain undiagnosed due to the lack of clinical suspicion and the absence of pathognomonic clinical and radiological characteristics. Because it is associated with a wide range of gynecological and obstetric complications, it is vital for healthcare providers to be aware of its existence and the role of antenatal radiological investigations in its diagnosis. The presence of multiple congenital abnormalities and a history of infertility in a pregnant woman should warrant the exclusion of müllerian duct anomalies from the beginning. Early detection of müllerian duct anomalies can facilitate an appropriate delivery plan and improve the general obstetric outcome.

A Clinicostatistical Study of Congenital Alimentary Tract Obstruction

Clinicostatistical datsa on 314 patients with congenital alimentary tract obstruction at the Department of Pediatrics and Pediatric Surgery of Seoul National University Hospital from January, 1974 to July, 1980 are presented and reviewed. The results are as follows; 1. Congenital megacolon(133 cases) is the most common congenital alimentary tract obstruction, and anorectal malformations(127 cases), congental duodenal obstruction(20 cass), esophageal atresia(12 cases), ileal atresia(9 cases), multiple intestinal atresia(2 cases), and colon atresia(2 cases), in given order of frequency. 2. The 20 cases of congenital duodenal obstruction included 15 male nad 5 female patients. The 10 cases were intrinsic obstruction(diaphragm of web; 4, atresia; 3, stenosis; 2, uncertain; 1) and the 10 cases were extrinsic obstruction(malrotation with or without Ladd's band, midgut volvulus: 9, preduodenal portal vein; 1). Associated anomalies were present in 3(1%) of the total. Operation was carried out in 19 patients and seven of them died. 3. The 22 cases of ileo-jejuno-colon atresia and stenosis included 14 male and 4 female patients. The majority of the patients involved ileum(9 cases), jejunum(9 cases), Jejunoileum(2 cases), and colon aresia was present in 2 cases. Associate anomalies were present in 7(32%) of the total. Operation was performed in 20 patients and seven of them died. 4. The 133 cases of congenital megacolon included 99 male and 34 female patients. The 121 case(91%) were short segment involvement and the remainders were long segment involovement. The 10 cases(8%) were associated with other malformations. In 118 patients operation was performed and the remainders were received conservative treatments, including enema. The 20 cases, 18% of the patients developed complications such as enterocolitis, incontinence, and so on. Three patients died during follow-up.

A Clinicostatistical Study of Congenital Alimentary Tract Obstruction

Clinicostatistical datsa on 314 patients with congenital alimentary tract obstruction at the Department of Pediatrics and Pediatric Surgery of Seoul National University Hospital from January, 1974 to July, 1980 are presented and reviewed. The results are as follows; 1. Congenital megacolon(133 cases) is the most common congenital alimentary tract obstruction, and anorectal malformations(127 cases), congental duodenal obstruction(20 cass), esophageal atresia(12 cases), ileal atresia(9 cases), multiple intestinal atresia(2 cases), and colon atresia(2 cases), in given order of frequency. 2. The 20 cases of congenital duodenal obstruction included 15 male nad 5 female patients. The 10 cases were intrinsic obstruction(diaphragm of web; 4, atresia; 3, stenosis; 2, uncertain; 1) and the 10 cases were extrinsic obstruction(malrotation with or without Ladd's band, midgut volvulus: 9, preduodenal portal vein; 1). Associated anomalies were present in 3(1%) of the total. Operation was carried out in 19 patients and seven of them died. 3. The 22 cases of ileo-jejuno-colon atresia and stenosis included 14 male and 4 female patients. The majority of the patients involved ileum(9 cases), jejunum(9 cases), Jejunoileum(2 cases), and colon aresia was present in 2 cases. Associate anomalies were present in 7(32%) of the total. Operation was performed in 20 patients and seven of them died. 4. The 133 cases of congenital megacolon included 99 male and 34 female patients. The 121 case(91%) were short segment involvement and the remainders were long segment involovement. The 10 cases(8%) were associated with other malformations. In 118 patients operation was performed and the remainders were received conservative treatments, including enema. The 20 cases, 18% of the patients developed complications such as enterocolitis, incontinence, and so on. Three patients died during follow-up.