Multiple-stage correction of caudal duplication syndrome: a case report

Caudal duplication syndrome is a very rare congenital deformity. A 13-year-old boy was born with duplicated colon-rectum and anus, diphallus, hydronephrosis of left kidney with megaureter, double bladders and urethras, and vertebral abnormalities. Multiple-stage correction was performed to remove the duplicated colon and the mucosa of the duplicated rectum. A new colon was reconstructed. The left kidney and megaureter were excised. The septum in the bladders was removed to convert the double bladders into a single bladder. The double phalluses were fused into a single penis. After these staged procedures, the boy is now living a normal life.     

Perineal mass protrusion with rectal mucosa: a rectal duplication that underwent exstrophy

We present a rare case of a male neonate with a perineal mass with rectal mucosa, diagnosed as an exstrophic duplication of the rectum. It was accompanied by a cord that was deeply invested in the pelvic diaphragm and was composed of smooth muscle, fibrous tissue, and some rectal glands. The association of exstrophic rectal duplication with a bifid scrotum, hypospadias, and normal anus has not been described previously in the literature.     

应用国产双吻合器行直肠癌保肛手术

目的探讨国产双吻合器在直肠癌保肛手术中的应用价值。方法回顾性总结我院1996年以来67例直肠癌患者应用国产双吻合器法行结直肠吻合保肛手术的经验。结果本组在直肠癌手术中应用双吻合器作直肠残端闭合均成功。吻合器切割不全4例,术后发生吻合口瘘2例(3.0%),切口感染2例(3.0%),吻合口出血1例(1.5%)。术后病理检查切缘癌细胞阴性65例,阳性2例。术后局部复发3例(4.5%),无手术死亡。结论国产双吻合器在低位直肠癌保肛手术中是安全可靠的,它可以完成以往手法缝合难以完成的低位结直肠吻合术。     

低位直肠癌保肛手术60例治疗体会

目的探讨直肠全系膜切除联合双器械吻合在低位直肠癌保肛术中应用效果及其实用性和安全性。方法回顾性分析2010年1月至2013年12月我院60例用全直肠系膜切除加双吻合器行保肛根治手术的临床资料。结果 60例均保肛成功,无手术死亡病例(其中32例行预防性造瘘术)。术后共发生并发症8例(13.3%),吻合口漏2例(3.3%),均为未行预防性造瘘术患者。所有并发症经保守治疗后均治愈。术后一年内局部复发3例(5%)。结论全直肠系膜切除联合双器械吻合行低位直肠癌保肛术不仅提高了保肛率且降低了局部复发率,是一种安全有效的手术方式。     

Complete duplication of the bladder, urethra and external genitalia in a male neonate with an imperforate anus

A rare case of a male neonate with complete duplication of the bladder, urethra and external genitalia with associated anomalies is reported. In addition to the above, the patient had a hypoplastic left kidney, bilateral bifid scrotums (four hemi-scrotums), an imperforate anus and a mass on the perineum.     

Tubular duplication of the hindgut with genitourinary anomalies.

Hindgut duplication including the colon and rectum as well as the genital and urinary organs are extremely rare. Only a few cases are noted in the medical literature. In this report, a newborn with exstrophy of the urinary bladder, double vagina and uterus, double anus, and complete duplication of rectum and colon with malrotation is presented.     

Imperforate anus, malrotation, and Hirschsprung's disease with double zonal aganglionosis: an extremely rare combination

The author describes an extremely rare case of coexistence with imperforate anus, malrotation, and double zonal aganglionosis.A colostomy was performed on a 2-day-old male infant at the proximal sigmoid colon, because the finding of an invertgram revealed an intermediate type of imperforate anus. At the age of 1 month, a distal colostogram showed the low type without fistula. An upper gastrointestinal series revealed malrotation. At 4 months of age, Ladd's procedure and an anoplasty were done. The function of the colostomy was not good. At laparotomy, a narrowing terminal ileum was removed for being a suspected intestinal obstruction. Histologic findings of the removed ileum revealed aganglionosis. A rectal suction biopsy showed the positive finding of acetyl cholinesterase staining. Therefore, the patient was diagnosed with extensive aganglionosis. At 9 months of age, a 1-stage ileoendorectal pull-through with a right colon onlay patch was performed. Histologically, a skipped ganglionic bowel segment at the right colon, a double zonal aganglionosis, was found among the extensive aganglionosis. Although the ganglionic right colon was used for the colon patch, which was placed for antiperistaltic movement, postoperatively the bowel function was excellent.The author herein describes an extremely rare case of coexistence of imperforate anus, intestinal malrotation, and double zonal Hirschsprung's disease. A case with this association, to the author's knowledge, has not been reported previously in the literature.     

Ruptured rectal duplication cyst with classical bladder exstrophy

A newborn boy was brought to us, 2 hours after birth, with a mucosal-lined left hemiperineal lesion associated with classical bladder exstrophy and an anterolaterally displaced anus. Perineal anatomy was restored by excising the mucosa lined lesion. The bladder closure for classical bladder exstrophy was done at the same time. Histologically, gastric, respiratory, and small intestinal epithelia were present in the mucosa. A rectal duplication cyst that had ruptured in utero through the hemiperineum could explain the anomaly. The association of classical bladder exstrophy with ruptured rectal duplication cyst has never previously been described in the literature.     

Diagnosis and management of congenital rectourethral fistula in a child with long tubular duplication of the colon and Klippel-Feil syndrome

We describe a unique association of congenital rectourethral fistula with long tubular duplication of the colon in a boy with Klippel-Feil syndrome and Sprengel deformity. He presented with a rectourethral fistula after surgical repair of a tubular duplication of the terminal ileum, colon, and proximal rectum. Preoperative identification of the fistula was challenging and was only achieved after cystoscopy with injection of methylene blue under pressure through Foley catheters placed into the anus and distal stoma of a colostomy. Surgical repair was performed through the posterior sagittal approach. The patient is doing well after 4 years of follow-up.     

下段直肠癌双重器械吻合保肛手术49例分析

目的 总结下段直肠癌双重器械吻合保肛手术经验.方法回顾性分析49例下段直肠癌用双重器械吻合手术及术后随访资料.结果 49例中无手术死亡,术中快速活检,3例远端阳性再切除1cm以上,术后病检,直肠切除后两端均无肿瘤残留.3例盆腔感染,2例切口感染经冲洗、换药等治愈.术后均用多药联合化疗,随访1、3、5年,生存率分别100%、85.7%、61.2%.结论下段直肠癌用双重器械吻合保肛手术临床效果好.     

U形肌袢成型重建原位肛门治疗低位直肠癌和肛管癌

目的在Miles术式的基础上,采用U形肌袢成型重建原位肛门治疗低位直肠癌和肛管癌。方法为72例低位直肠癌和肛管癌患者实施保留耻骨直肠肌双重U形肌袢成型重建原位肛门,同时建立直肠角和直肠瓣的新术式。结果本组72例,于术后4~6d开始出现便意感,1个月左右排便有自控意识,排便次数1~2次/日。1年内,优41例,良19例,差12例。结论本术式是在Miles术式基础上完成重建肛门,能彻底切除肿瘤,重建肛门排便功能良好,生活质量满意。     

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.     

Duplication of pouch colon associated with duplication of the lower genitourinary tract

The authors report on a baby with imperforate anus associated with duplication of descending colon, double pouch colon type IV, duplication of the urinary bladder, and a bifid penis. The interesting presentation of this problem and its management is discussed with a brief review of the probable embryologic basis for such an anomaly. Such a duplication of pouch colon has not been described previously. J Pediatr Surg 38:E1.     

Spine duplication or split notochord syndrome – case report and literature review

Context: Spine duplication is a rare condition, with various extents and severe additional anomalies. The goal of this study was to describe a unique case of a boy with split notochord syndrome who was followed up from birth until maturity.

Findings: Physical examination at birth showed defects of the abdominal wall and cloacal exstrophy with visible urether outlets. A transposed anus was present in the perineal region. Split bony elements of the spine with nonpalpable sacral bone were noted. A soft, skin-covered lump, with the consistency of a lipoma, was present in the sacral area. There was asymmetry of the lower limbs: the left was hypoplastic, with a deformed foot and hip. Computed tomography revealed a normal shape of the Th12 and L1 vertebrae, whereas the L2 was split. Downward from L3, there were two vertebrae at each level, with two spinal canals. The spinal cord divided into two “semicords” at the level of L1. Neurologic status and the shape of the spine remained unchanged during puberty. The last follow-up was performed at the age of 18 years. He managed to walk independently in prosthesis with visible limping.

Conclusion: Spine deformities are always suspected in neonates with lipoma in the sacral region, which may sometimes be serious. Walking ability and quality of life depend on neurologic deficits; even with long duplication and double sacrum, walking can be a feasible option.     

凯途(CONTOUR)在低位直肠癌双吻合器技术中的应用

目的:评价CONTOUR在双吻合器技术中的作用.方法:回顾性分析2005年4月至2006年7月在低位直肠癌中应用的90例CONTOUR和219例XF进行双吻合器技术的比较.结果:CONTOUR组保肛率(57.8%)优于XF组(44.7%);吻合口漏、吻合口狭窄和吻合口出血等并发症两组无差异.结论:CONTOUR不增加并发症,能提高保肛率,是双吻合器技术中XF很好的替代产品.     

Primary laparoscopic repair of high imperforate anus in neonatal males

Purpose

The standard approach to males with high imperforate anus has been a staged procedure starting with a descending colostomy, then posterior sagittal anorectoplasty with colostomy closure after 3 months. Recently, a minimally invasive approach to the repair of high imperforate anus has been described in infants after colostomy. We describe 6 newborn males with high imperforate anus successfully repaired laparoscopically as a primary, single-stage procedure.

Methods

A retrospective chart review was performed on all patients with imperforate anus from October 2003 to October 2006.

Results

We evaluated 9 newborn males with high imperforate anus. Of these patients, 6 underwent primary laparoscopic repair on day 1 to day 2 of life. Of these 6 patients, 3 were found to have bladder neck fistulas, whereas the other 3 had prostatic urethra fistulas. All patients passed stool within the first 72 hours postoperatively. One patient has required a procedure for a mild rectal prolapse. Follow-up ranges from 2 to 30 months in the single-stage group.

Conclusion

Our early results using primary laparoscopic repair appear encouraging. Laparoscopy allows excellent visualization and assessment of the fistula and repair of high imperforate anus without need for colostomy. Long-term follow-up will be needed to assess outcomes and continence rates.     

Recurrent epididymo-orchitis caused by posterior urethral valve associated with imperforate anus

Epididymo-orchitis is an uncommon complication of imperforate anus. A 14-year-old boy who received surgical repair for imperforate anus was diagnosed with right epididymo-orchitis. Intravenous pyelography showed a right solitary kidney. Posterior urethral valve, reflux of contrast medium to the ejaculatory duct and incomplete duplicate urethra were suspected from voiding cystourethrography. Valve ablation was performed to prevent the recurrence of epididymo-orchitis. Improvement of urinary force was achieved and the patient has been free of recurrence during a 2-year follow up. For the patient with imperforate anus, we should evaluate not only anorectal function, but also the genitourinary tract in order to preserve renal function and fertility.     

会阴肛门成形术治疗先天性无肛19例临床分析

目的分析和评估会阴肛门成形术治疗先天性中低位无肛患儿的疗效。方法回顾性分析2003年10月至2010年8月我科收治的19例中低位无肛畸形儿采用会阴肛门成形术治疗的临床资料。结果术后随访3个月至3年,19例患儿肛门功能及排便功能正常。结论会阴肛门成形术治疗中低位无肛疗效是确切的,值得临床推广。     

低位直肠前切除术应用双重器械吻合的经验

目的：探讨应用双重器械吻合在保留肛门手术中的应用。方法：回顾性分析我们1994年6月至2002年1月期间对114例低位直肠癌病人应用双重器械吻合，行低位直肠前切除术的临床资料及经验。结果：本组114例无手术死亡，4例（3．5％）术后出现吻合口漏，5例（4．4％）发生吻合口狭窄，经冲洗引流及扩肛2个月后皆治愈。结论：对于低位直肠癌的保肛手术，应用双重器械吻合可使手术简便、可靠、安全、省时。     

Complete duplication of the hindgut and lower urinary tract with diphallus

An infant is reported with complete duplication of the colon, rectum, anus, terminal ileum up to the point of Meckel's diverticulum, doubling of the genitalia with completely formed penes, double bladder and urethra, multiple spinal anomalies, omphalocele, and large lower abdominal wall hernia with wide separation of the symphysis pubis.