Alimentary tract duplications in children: report of 26 years' experience

Duplications of the alimentary tract are one of the rare anomalies of the gastrointestinal system. Because of the wide spectrum of the signs and symptoms, preoperative diagnosis frequently cannot be made. A close familiarity with clinical and surgical characteristics provides appropriate management and treatment of duplications. A retrospective clinical study was conducted to evaluate clinical and surgical characteristics and the treatment of duplications of the alimentary tract. During a 26-year period between 1971 and 1997, 38 patients with duplications of alimentary tract underwent operation at the Hacettepe University Department of Pediatric Surgery. Forty-two duplications in 38 patients (20 male, 53%; 18 female, 47%) were encountered. Sixty-nine percent of the patients were symptomatic under the age of one year, with 24 percent presenting with symptoms in the neonatal period. There were one sublingual, nine intrathoracic (including 2 thoracoabdominal) and 32 intraabdominal duplications. Abdominal mass, abdominal distention, constipation, vomiting and respiratory distress were the most frequently encountered signs and symptoms. Plain thoracic and abdominal X-rays, ultrasonography, and computed tomography of the chest and abdomen were the most commonly used diagnostic radiological methods. Thirty-three duplications (79%) were spherical and nine (21%) were tubular. Multiple duplications were encountered in two patients (5.3%). Fourteen duplications (33%) contained heterotopic mucosa, mostly gastric type. More than one type of heterotopic mucosa in the same duplication was encountered in four duplications (10%). Additional malformations were encountered in 26 percent of patients. Six patients (15.8%) died from unrelated causes. The signs and symptoms vary among duplications. Signs and symptoms leading to diagnosis and surgery varied according to the age of patient, location of the duplication, type of mucosal lining, duration of disease and presence of complication. The ideal surgical treatment of duplication is complete excision. However, the other treatment options should be well known.     

Esophageal duplication in children. Report of 7 cases]

Esophageal duplications represent 10 to 20% of esophageal malformations and 15 to 20% of digestive duplications. METHODS: The authors report a series of seven cases observed between 1985 and 1999. RESULTS: The sex ratio was 2.5. The diagnosis was made before the age of six months in five cases. The clinical presentation included respiratory signs in six cases and digestive signs in five cases. The diagnosis was made on the association of a mediastinal mass behind vertebral malformation in five cases. The duplication was cystic in five cases and tubular in two. A perforation in the pleura occurred in one case of tubular duplication. The intervention consisted of total excision of the duplication in four cases and subtotal resection in two cases. For the seventh patient, who presented a perforation of the duplication in the pleura, the operation comprised a thoracic drainage associated to feeding gastrostomy. The histologic examination of the six operative specimens confirmed the diagnosis of esophageal duplication. Six patients survived and have favorable evolution with an average follow-up of four years. DISCUSSION: Esophageal duplications represent 15 to 20% of digestive duplications. Their clinical presentations are variable. Their diagnosis is often made before the age of two years, when a posterior mediastinal mass is associated with vertebral malformations. Apart from the tubular form, which is rare, diagnosis is confirmed by the histologic study of the operative specimen. CONCLUSION: Esophageal duplications are rare and often pose a problem of positive diagnosis. Their treatment is surgical and should be undertaken before the appearance of complications.     

消化道重复畸形12例

目的探讨消化道重复畸形的临床和病理特点。方法回顾性分析消化道重复畸形共12例。男8例,女4例。收集临床资料,对患儿进行随访,复习病理切片并结合临床分析。结果本组12例均经手术及病理检查证实诊断,重复畸形解剖部位分布:回盲部4例,食管、结肠各2例,胃、空肠、直肠、肛管各1例。术后均恢复正常。10例重复畸形黏膜性质与主肠管相似,2例临床表现为便血、贫血者在病理上均为异位胃黏膜组织。结论手术切除是治疗消化道重复畸形的最好选择。     

Duodenal duplications. Clinical characteristics, embryological hypotheses, histological findings, treatment.

BACKGROUND: The aim of this study was to analyse different clinical aspects and embryologic hypotheses of duodenal duplications. METHODS: Duodenal duplications occurring since 1995 were recorded. The age of the children at the time of diagnosis, the sex, location of the duplication, type of mucosa, clinical signs, associated lesions, and the type of surgical intervention were defined. RESULTS: We identified 5 patients (3 girls and 2 boys) who presented with histological or intraoperative findings of duodenal duplication. Their ages ranged from 4 days to 9 years, with the exception of two prenatal diagnoses. Three children were symptomatic: high intestinal obstruction (1 case), digestive bleeding (2 cases). In 1 case we found a palpable abdominal mass and 1 case was completely asymptomatic (prenatal detection). The abnormality was located on the duodenal concavity, originating from the third part in 2 cases and from the second part in 3 cases. All cases were non communicating types, 4 of which were cystic duplications and 1 was a tube-like variety. The epithelial lining was duodenal mucosa in all patients, but gastric heterotopies were identified in 2 cases. We performed two complete resections and 3 intraduodenal derivations. The outcome was uneventful in 5 cases with an average follow-up of 2 years. CONCLUSION: Duodenal duplications are rare malformations with several anatomical varieties. The preferred treatment for duodenal duplications is complete removal when the location allows it without endangering nearby anatomical structures.     

Delayed presentation of congenital diaphragmatic hernia

The late-presenting congenital diaphragmatic hernia (CDH) represents a considerable diagnostic challenge. This study was undertaken to define various patterns of delayed presentation and to analyze pitfalls in the diagnosis and treatment of these patients. Thirty-three children with CDH were treated between 1993 and 2000; 15 of these (45.5%) who were diagnosed after the age of 2 months-14 years, median of 2.5 years are reported. Thirteen had a Bochdalek hernia and 2 had a Morgagni hernia. The diaphragmatic defect was right-sided in 6 cases and left-sided in 9. Five patients presented acutely, 3 with respiratory distress and 2 with gastrointestinal (GI) obstruction. The remaining 10 presented with chronic respiratory or GI complaints. Inappropriate insertion of a chest drain occurred in 3 patients misdiagnosed as having pleural effusion (2) and a pneumothorax (1). Two patients had previous chest radiographs reported normal. Plain radiographs were sufficient to make a definitive diagnosis in only 6 patients; GI contrast studies were necessary in the other 9. All patients were treated through an abdominal approach with primary closure of the diaphragmatic defect without a patch. A distinct hernia sac was present in 6 cases, and associated malrotation in 6. All except 1 patient survived the operation with rapid improvement of their GI and respiratory symptoms. We conclude that: (1) late-presenting CDH should be included in the differential diagnosis of any child with persistent GI or respiratory problems associated with an abnormal chest X-ray film; (2) nasogastric tube placement must be considered as an early diagnostic or therapeutic intervention when the diagnosis is suspected; and (3) GI contrast studies should be a part of the diagnostic work-up of these patients.     

Anterior colorectal duplication presenting as rectal prolapse

Duplications of the gastrointestinal (GI) tract are rare. Only 5% of them are rectal and there are very few reports of rectal prolapse (RP) caused by a duplication. An 11 month-old female presented with a RP caused by a blind-ended anterior tubular colorectal duplication. The duplication was successfully opened and connected to the normal rectum without complications. Although infrequent, a rectal duplication should be considered in the differential diagnosis of RP.     

Rectal duplications in children: two case reports and review of the literature

Duplications of the rectum are extremely rare, with only 60 cases reported in the world literature. We report two patients with cystic duplications of the rectum who underwent surgical intervention in our institution during the past 10 years. A 5-year-old white male presented with constipation and tenesmus as well as a 4-cm cystic mass that protruded from the anus when he strained to pass stool. Rectal examination and ultrasound suggested the clinical diagnosis of rectal duplication. Transanal submucosal resection of a 3-cm cystic mass adherent to the posterior rectal wall was carried out. Histologic examination revealed a rectal duplication containing zones of ciliated respiratory epithelium. The second patient, a 6-year-old white female, presented with a history of chronic constipation. A large, cystic presacral mass was detected by rectal examination and confirmed by CT scan. Complete submucosal resection of the cystic duplication was performed through a posterior trans-sacral approach. Histology revealed a rectal duplication lined by respiratory epithelium. Although duplications are generally lined by mucosa of the adjacent bowel, heterotopic mucosa, most commonly gastric or pancreatic, may be present. Rectal duplications with ectopic gastric mucosa have been previously described, but to our knowledge the presence of respiratory epithelium in a rectal duplication has never been reported. Complete resection of these lesions results in cure.Presented at the 21st Annual Meeting Canadian Association of Paediatric Surgeons Edmonton, Alberta, Canada September 21–23, 1989 Offprint requests to: A. L. Bensousson     

Alimentary tract duplications in infants and children.

This is a review of 30 duplications of the alimentary tract in 28 patients treated at the Surgical Unit of the Children's Department of the Medical University of Pécs, Hungary, and at the Department of Pediatric Surgery of the Medical Academy of Dresden, Germany, from 1964 to 1989. The ages of patients ranged from 1 day to 13 years, 80 percent were less than 2 years of age at initial presentation. There were 6 thoracic, 20 abdominal and 2 thoraco-abdominal duplications. Distended abdomen, vomiting, bowel obstruction and palpable abdominal mass were most frequently encountered. Plain thoracic and abdominal x-rays, ultrasonography, barium esophagogram, barium meal and enema were the most common diagnostic procedures. Emergency operative intervention was required in 18 patients. One infant died of an unrelated disease. Twenty-three duplications were cystic and 3 tubular. One patient had an appendiceal duplication, and another patient a flat lumenless duplication located on the perineum close to the anal opening. The surgical procedure--removal of the duplication--should not be more radical than necessary to eliminate the potential complaints and prevent recurrence. During surgery the common blood supply shared between the duplication and the native bowel must be carefully protected to avoid undue sacrifice of normal bowel.     

Complex colon duplication mimicking an obstructed,non-functioning kidney in a newborn with imperforate anus and spinal dysraphism

Gastrointestinal (GI) duplications contain tissue resembling several portions of the GI tract and are associated with vertebral and genitourinary (GU) abnormalities [1–4]. We report a newborn with low, imperforate anus and lumbosacral dysraphism, who presented with a large cystic mass in the left renal fossa and pelvis. The flank mass (felt initially to be a dysplastic kidney and ureter) proved to be a complex GI duplication with histologic evidence of gastric, small bowel, and colonic mucosa, as well as respiratory epithelium and pancreatic tissue.     

Urethral duplication in males: our experience in ten cases

Urethral duplication is a rare congenital anomaly, affecting mainly boys. Clinical presentation varies because of the different anatomical patterns of this abnormality. We report our experience in ten males affected by urethral duplication. We retrospectively reviewed the records of ten males affected by urethral duplication. Mild cases of distal type I duplications as well as “Y-type” duplication associated to anorectal malformation were excluded. Evaluation included voiding cystourethrography, retrograde urethrography, intravenous urography and urethrocystoscopy. Mean age at diagnosis was 46.7 ± 32.3 months A blind ending duplicated urethra (type I) was present in three patients, two urethras originating from a common bladder neck (type II A2) in three, an “Y-type” duplication in three and a complete bladder with incomplete urethral duplication in one. Surgical management included excision of the duplicated urethra in four patients while a displacement of the ventral urethra (in “Y-type” duplication) in perineal-scrotal or scrotal position was performed in two patients as first stage of urethral reconstruction. Good cosmetical and functional results were achieved in all six treated boys while surgical management was not required in four. Urethral duplication is often associated with genito-urinary and gastro-intestinal abnormalities. Embryology is unclear and a lot of hypotheses have been proposed. We believe that the same embryological explanation cannot be applied to all subtypes of urethral duplication. Management must be evaluated for each case. The overall prognosis is good, in spite of the presence of other severe associate congenital anomalies.     

Intestinal obstruction caused by duplication of the caecum

Two cases of caecal duplication are presented, one in a neonate and one in an infant. The diagnosis was made at laparotomy, which had been undertaken for the presumptive diagnosis of intestinal atresia and torsion of an ovarian cyst respectively. Also the literature on alimentary tract duplications is reviewed, referring to the incidence, presenting symptoms, and location of the duplication, in particular that of the caecum.     

Perforated tubular duplication of the transverse colon: a rare cause of meconium peritonitis with prenatal diagnosis

The transverse colon is an exceptional location of intestinal duplication. Perforated duplications are rarely described in neonates. Meconium peritonitis (MP) can originate from prenatal perforated intestinal duplication. The authors report a case of a baby girl with prenatal diagnosis of MP. Rapid worsening of clinical aspects at birth and the presence of a pneumoperitoneum on systematic abdominal plain radiographs led to urgent surgery on the 1st day of life. Laparotomy showed a perforated necrotizing tubular duplication of the transverse colon. Removal of the duplication followed by limited segmental colonic resection and double colostomy were carried out. Follow-up was uneventful.     

Multiple short-segment colonic duplications

Gastrointestinal tract duplication is an uncommon congenital anomaly that rarely involves the colon. We report an unusual case of multiple short-segment duplications of the colon not previously reported in the literature. The patient had a large diverticulum-type duplication of the descending and sigmoid colon and two other tubular duplications of the transverse and ascending colon, the latter two communicating with the colon at both ends of the duplications. The symptomatic diverticulum-type of duplication was surgically treated.     

Outcome in juvenile dermatomyositis

The clinical features, outcome and complications of juvenile dermatomyositis were studied in a tertiary care hospital by retrospective analysis of case records. Nineteen patients were treated over an 11-year period. Median age at diagnosis was 12 years (2.5-16 years). Median duration of disease prior to diagnosis was 12 months (2-96 months). Proximal muscle weakness was seen in all 19 cases, neck muscle weakness in 14, pharyngeal muscle involvement in 5 and respiratory muscle involvement in 3 cases. Heliotrope rash was seen in 9 and Gottrons rash in 8 patients. Myocarditis and GI bleed were seen in 1 each while interstitial lung disease was seen in 2 patients. All except one patient received prednisolone. Methotrexate was used in 13 and azathioprine in 3 patients. Eight patients are in complete remission (CR), 8 partial remission and 2 patients had no response. Complications were calcinosis in 5, contractures in 2, TB in 4 and pyogenic infections in 4 patients. Juvenile dermatomyositis needs to be recognised early and treated aggressively to improve outcome.     

小儿胃重复畸形的诊治

目的 探讨小儿胃重复畸形在临床表现、诊断及治疗上的特点,以期减少误诊,提高诊治水平.方法 回顾分析2010年3月至2015年3月我院收治的4例胃重复畸形患儿诊治过程.其中女3例,男1例.年龄9个月至11岁,术前B型超声或CT检查均发现上腹部囊性肿块,但不能明确肿块来源,经腹腔镜探查及开腹探查术中确诊.不同类型胃重复畸形可行不同方式切除.结果 腹腔镜辅助脐孔拖出切除2例,完全腹腔镜下切除1例,中转开腹手术切除1例.手术顺利,术后无并发症出现,分别随访1个月、1年、2年、3年,术前症状完全消失,恢复良好.结论 小儿胃重复畸形较为少见,无特异性临床表现.腹部B型超声及CT易于发现病变,常为上腹部囊性肿块,但难以分辨病变的来源,常误诊为胰腺囊肿、肠重复畸形等病变.外科手术探查是诊断胃重复畸形的有效手段,不复杂的囊状胃重复畸形可选择腹腔镜辅助或单纯腹腔镜切除,单纯重复畸形体切除能达到较好治疗效果.     

Surgical complications of the haemolytic uraemic syndrome

During the first outbreak of haemolytic uraemic syndrome (HUS) to be reported in Australia, 22 children were admitted to the Women's and Children's Hospital, Adelaide. The outbreak was caused by an entero-haemorrhagic Escherichia coli strain (EHEC) of serotype 011:H-, a strain rarely implicated as a cause for HUS. In all 22 patients, the onset of HUS was preceded by a gastrointestinal (GI) prodrome. All patients had diarrhoea. In 17 (73%), the diarrhoea became bloody; in 20 (86%) there was vomiting; in 15 (65%) there was abdominal pain; and in 12 (50%) all three symptoms were present. Abdominal symptoms continued to complicate the course of 4 patients. Two of these underwent exploratory laparotomy, both had gangrenous colon excised, and both survived. The 2 remaining patients were successfully treated non-operatively. One further patient underwent appendicectomy before the diagnosis of HUS was made. There was 1 death during this epidemic. In patients with HUS and GI involvement, optimal surgical management requires careful consideration of the indications for, and the timing of, surgical intervention.     

Type 1 rectal duplication: associated congenital malformations and ultrasound diagnosis

Type I duplication of the rectum is a rare condition that in most reported cases does not occur in association with other congenital malformations. We describe three cases, two of which had duplications of other structures: the first had a functioning supernumerary pelvic kidney, the second a duplication of the bladder, urethra, and vagina; the third had no associated malformations but was unusual, because the duplication cyst lay anterior to the rectum, and unique in that the diagnosis of an intra-abdominal cyst was made by ultrasonic examination. Correspondence to: G. M. A. Hendry     

Anatomical Investigation of 39 Cases of Congenital Duplication in Calves

Abstract The anatomical features of congenital duplication in the bovine calf encountered in 39 cases over eleven years in Hokkaido were investigated macroscopically. Among the animals studied, 14 were male, 20 female and 5 of unknown gender, and the anomaly was noted in 35 Holstein-Friesians, 3 Japanese Blacks and 1 Hereford.
The duplications observed in this study were classified by four types: free asymmetrical, 4 (all 4 acardius); attached symmetrical, 25 (14 cranial duplication, 2 dipygus, 2 dicephalus dipygus, 5 thoracopagus, and 2 pygopagus); attached asymmetrical, 6 (1 parasitic dipygus, 4 notomelia, and 1 pygomelia); and miscellaneous, 4. The four acardii were holoacardii amorphi covered with skin and hair. Cranial duplication was subdivided into five types based on the number of eyes and ears. In symmetrical twins the anterior part of the body was affected in 16 out of 25 cases (64%), the posterior part in 2 out of 25 (8%) and both the anterior and posterior in 7 out of 25 (28%). All were mirror-image duplicates on the various planes, and in some the internal organs had a center of symmetry. All four notomeli calves were female. Among the miscellaneous duplications, three were of the genital organs (male 1, and female 2) and one was a spinal cord duplication.     

Malrotation in Children with Symptoms of Gastrointestinal Allergy and Psychosomatic Abdominal Pain

Three children with malrotation 4.5, 5 and 9 years old at operation are presented. Their preliminary diagnoses were gastrointestinal (GI) allergy, GI allergy with colon irritabile and psychosomatic abdominal pain. They were treated on an outpatient basis under these diagnoses for more than two years before their malrotations were discovered. In two children radiology did not demonstrate any signs of intestinal obstruction and in one of these children repeated radiological examinations were necessary for the diagnosis of malrotation. Operative findings were chronic volvulus with compromised blood flow and obstructive duodenal bands. The third child suffered acute strangulation ileus with gangrene of the intestines before diagnosis was made. We conclude that special consideration must be given to the possibility of malrotation when treating and radiologically investigating children with GI disturbances.     

Malrotation in children with symptoms of gastrointestinal allergy and psychosomatic abdominal pain

Three children with malrotation 4.5, 5 and 9 years old at operation are presented. Their preliminary diagnoses were gastrointestinal (GI) allergy, GI allergy with colon irritable and psychosomatic abdominal pain. They were treated on an outpatient basis under these diagnoses for more than two years before their malrotations were discovered. In two children radiology did not demonstrate any signs of intestinal obstruction and in one of these children repeated radiological examinations were necessary for the diagnosis of malrotation. Operative findings were chronic volvulus with compromised blood flow and obstructive duodenal bands. The third child suffered acute strangulation ileus with gangrene of the intestines before diagnosis was made. We conclude that special consideration must be given to the possibility of malrotation when treating and radiologically investigating children with GI disturbances.