Solitary intestinal fibromatosis: a rare cause of neonatal obstruction

Solitary intestinal fibromatosis (SIF) is an unusual cause of neonatal intestinal obstruction. A fibromatosis located in the first part of the duodenum, close to the pylorus, was associated with a focus of heterotopic pancreas and mimicked congenital pyloric stenosis. It illustrates the difficulties encountered in the diagnosis and surgical management of this condition.     

Solitary Intestinal Fibromatosis: a Rare Cause of Neonatal Obstruction

Solitary intestinal fibromatosis (SIF) is an unusual cause of neonatal intestinal obstruction. A fibromatosis located in the first part of the duodenum, close to the pylorus, was associated with a focus of heterotopic pancreas and mimicked congenital pyloric stenosis. It illustrates the difficulties encountered in the diagnosis and surgical management of this condition.     

Neonatal intestinal obstruction from solitary intestinal fibromatosis

 Solitary intestinal fibromatosis (SIF) is a very rare condition, with only 13 cases reported. We present a new case of SIF causing neonatal intestinal obstruction and review the literature on this condition. SIF appears to be a condition of infancy and carries a very good prognosis after segmental resection. Accepted: 20 March 2000     

Mesenteric fibromatosis in childhood

A case of omento-mesenterial fibromatosis in a 6-year-old boy revealing through acute intestinal obstruction is reported. Mesenterial fibromatosis, also known as aggressive fibromatosis or desmoid tumor is generally associated with Gardner's syndrome but otherwise an extremely rare disease. Fibromatoses do not metastasize but are characterized by a high incidence of local recurrence. Primary treatment is operative. After multiple recurrence irradiation, cytostatics and antiestrogens have been used additionally to control the disease.     

Colonic stricture secondary to torsion of an ovarian cyst

Intestinal obstruction in the newborn is a potentially life-threatening complication. The most common causes are meconium plug, meconium ileus, intestinal atresia, intestinal malrotation, and Hirschprung's disease. We present an unusual case of intestinal obstruction caused by torsion of an ovarian cyst. The left fimbria and ovary swirled around the sigmoid colon, causing colonic stricture.     

Intraoperative colonic lavage in infants using a Replogle tube

On-table colonic lavage for preparing obstructed bowel prior to anastomosis or stoma formation is now routine in adults, but it is seldom used in paediatric practice. We describe a simple technique for intraoperative colonic lavage using inexpensive medical devices that are also readily available and disposable, a Replogle tube, a fine-bore sucker, and a 20-ml syringe. We have used this technique in infants with intestinal obstruction due to anorectal malformations, intestinal atresias, and post-NEC strictures, to good effect.     

Solitary Intestinal Fibromatosis with Perinatal Bowel Obstruction

Three newborn male infants presented with bowel obstruction in the first day of life and at surgery were found to have solitary tumors involving the small or large intestine. Histologic examination in each case showed a transmural infiltrative spindle cell lesion having the morphologic features of fibromatosis. Ultrastructural studies in one case revealed the tumor to be composed of myofibroblasts. The patients are all alive and well without recurrences 26 months to 10 years after surgery. Only 3 previous cases of solitary congenital fibromatosis of the intestinal tract have been reported. Some of the other congenital spindle cell tumors cited in the literature under various names have morphologic and biologic similarities to our cases and may in fact be examples of congenital fibromatosis. The appropriate treatment of this unusual lesion is local excision, and the prognosis is excellent.     

Colonic diverticulitis causing partial bowel obstruction in a child with cystic fibrosis

Children and adolescents with cystic fibrosis (CF) may manifest bowel pathology with resulting bowel obstruction. Recognized causes of bowel obstruction in CF patients include meconium ileus, intussusception, distal intestinal obstruction syndrome and postoperative adhesions. Additionally, the development of colonic strictures in children with CF has recently been described. We report an unusual cause of partial obstruction of the ascending colon in a child with CF due to pathologically proven diverticulitis. Received: 17 March 1997 Accepted: 24 July 1997     

A sporadic abdominal desmoid tumour case presenting with intermittent intestinal obstruction.

Desmoid tumours, also known as aggressive fibromatosis, are rare lesions with an intermediate biological behaviour between benign fibrous lesions and fibrosarcomas. Although abdominal desmoids have an increased incidence in Gardner's syndrome, they are rarely found in an isolated form. We report the findings of a barium study, ultrasound, computed tomography and magnetic resonance imaging in a nine-year-old boy with intermittent nausea and vomiting, diagnosed as having a desmoid tumour. Although intra-abdominal desmoids are usually detected as a solitary lesion in sporadic cases, the presented case had two mesenteric lesions in the left upper quadrant. There was intestinal obstruction and the masses had invaded the colon wall.     

Gastroschisis with jejunal and colonic atresia,and isolated colonic atresia in dichorionic,diamniotic twins

Despite the increasing incidence of gastroschisis, the cause remains unknown. Genetic factors may contribute to bowel anomalies as demonstrated by cases of gastroschisis in twins and siblings, and other types of bowel anomalies in twins. Atresia of the colon represents one of the rarest causes of neonatal intestinal obstruction. We present the first case of dichorionic, diamniotic male twins in which there was gastroschisis with jejunal and colonic atresia in Twin A and isolated colonic atresia in Twin B.     

Hirschsprung’s disease complicating colonic atresia

A case of colonic atresia associated with Hirschsprung’s disease is described in a full term neonate presented with intestinal obstruction. Laparotomy revealed type III colonic atresia. Histopathological examination suggested total aganglionosis in the postatretic colonic segment. The child recovered satisfactorily following two stage Duhamel — Martin’s pull through procedure. Authors present their experience with the present case and the pertinent literature.     

Management of isolated and associated colonic atresia

Purpose  

Colonic atresia is a rare cause of congenital intestinal obstruction in the neonate. A review of the management and outcome of children with colonic atresia was performed.     

Intestinal atresia: management problems in a developing country

Over a 13-year period, 24 children with intestinal atresia were managed at the Jos University Teaching Hospital, Jos, Nigeria. Intestinal atresia ranks as the second most common cause of neonatal intestinal obstruction (after anorectal malformation) in our hospital. Five children had duodenal obstruction (two atresia, two duodenal webs, one annular pancreas), 17 had jejunoileal atresia, and two had colonic atresia. Fourteen were boys, and 10 were girls (M:F: 1.17:1). The median age at presentation to the surgeon was 6 days (range: 1 day-12 years). The most common presenting features were bilious vomiting and abdominal distension. Six patients did not pass meconium within the first 24 h of birth. The median weight at presentation was 2.6 kg (range: 1.1 kg-5.0 kg). Seven patients (four with jejunoileal atresia and three with duodenal obstruction) had associated congenital anomalies. Diagnostic investigation was plain abdominal x-ray, showing double-bubble gas shadows in duodenal atresia and varying degrees of air-fluid levels in jejunoileal and colonic atresias. An upper gastrointestinal series was done in three patients and a barium enema in one. Retrocolic duodenojejunostomy was done for all patients with duodenal atresia and annular pancreas, duodenotomy and web excision for those with duodenal webs, and resection with end-to-end anastomosis for those with jejunoileal atresia. One child with atresia involving the whole ileum and the colon had a jejunorectal anastomosis, while the other child with colonic atresia had caecostomy followed later by ileorectal anastomosis. Ten neonates died, giving a mortality rate of 41.7%. Mortality from intestinal atresia is still high in our environment, due mainly to lack of neonatal intensive care facilities.     

Uncommon co-occurrence of ileal atresia and total colonic aganglionosis in two unrelated newborns

We report two recent cases of distal ileal atresia associated with total colonic aganglionosis (TCA). It is well known that ileal atresia and Hirschsprung’s disease (HD) are individually frequent causes of intestinal obstruction. However, the association of both these diseases is an extremely rare event. To our knowledge, only 19 cases of ileal atresia associated with HD have been described so far. When a child is diagnosed having ileal atresia, the possibility of associated TCA should be considered. Therefore, intra-operative staged biopsies should be sent for histological examination in order to rule out or confirm this very rare co-occurrence.     

Hirschsprung's disease presenting as calcified fecaloma

Fecaloma, a discrete mass of intestinal debris and fecal material usually occurring in the colon, is a rare finding in children. The case of a six-year-old boy is presented who developed signs of partial colonic obstruction which was due to a large, calcified fecaloma of the rectosigmoid. Barium enema revealed changes indicating Hirschsprung's disease which were confirmed with rectal biopsy. Any child presenting with fecaloma should be evaluated for Hirschsprung's disease.     

The curious umbilicus: clue to the cause of abdominal pain

Omphalomesenteric duct derivatives cause an assortment of complications, most notably intestinal hemorrhage and obstruction. Intestinal obstruction is the most lethal complication and usually results from a diverticulum with an attachment to the umbilicus. This cause of intestinal obstruction is not generally recognized preoperatively. We report a teenager with episodic abdominal pain, acute small bowel obstruction, and a curious umbilical deformity with an underlying omphalomesenteric duct remnant. We believe that this physical finding suggests the diagnosis in patients with intestinal obstruction who have experienced unexplained episodes of abdominal pain.     

Congenital jejunoileal and colonic atresia

Jejunoileal and colonic atresias are a common cause of neonatal intestinal obstruction. Authors present the aetiology, types of atresias, clinical symptoms, diagnostics, surgical treatment, problems of postoperative management and prognosis in the newborns with congenital intestinal atresia. In the years 1992-2000, in the Department of Paediatric Surgery and in the Department of Pathology and Intensive Care of Newborn at the National Research Institute of Mother and Child in Warsaw, 539 newborns were surgically treated (congenital anomalies of gastrointestinal tract, genito-urinary system, central nervous system, defects of abdominal wall and diaphragm). In 21 patients jejunoileal or colonic atresia were confirmed. On the base of the course of treatment the authors analyse prognostic factors: prematurity, certain types of atresia, additional pathology accompanying intestinal defect, necessesity of re-section of the long part of the bowel or multiple anastomosis. Diagnostic and treatment problems in three patients with short bowel syndrome needed additional discussion.     

Solitary intestinal fibromatosis: a rare cause of intestinal perforation in neonates

Solitary intestinal fibromatosis (SIF) is very rare; only 9 cases have been described. A new case presenting unusually in a newborn is described and the previously reported cases are reviewed. SIF should be considered in the differential diagnosis of a newborn presenting with an intestinal perforation.     

胎粪性肠梗阻的临床研究

目的 探讨胎粪性肠梗阻的临床特点与治疗.方法 对4例胎粪性肠梗阻患儿的发病病因、临床症状、诊断及治疗进行回顾性分析.结果 4例胎粪性肠梗阻患儿以肠梗阻就诊3例,术前均怀疑为全结肠型无神经节细胞症,以消化道穿孔为首诊症状者1例,术前怀疑为新生儿坏死性小肠结肠炎.3例为新生儿期就诊,1例生后4个月就诊.4例患儿均进行手术治疗,其中2例行肠腔冲洗、末端回肠造瘘术,1例行回盲部狭窄肠管切除肠吻合术,1例行穿孔肠管修补术及腹腔冲洗引流术.3例新生儿患者术中均发现胎类极度黏稠、阻塞回盲部远端肠腔,4例患儿均在术中取病理证实肠壁存在神经节细胞.随访中1例患儿出院1个月后反复发生呼吸道感染、重症肺炎、呼吸衰竭而死亡:1例患儿X线胸片持续存在肺部阴影,但呼吸正常;其他2例患儿生长发育正常.结论 新生儿期便秘患儿在怀疑全结肠型无神经节细胞症的同时,应考虑到本病的可能,术中病理活检对诊断该病十分重要,术后随访应密切注意肺等其他器官系统的相应合并症.     

Preduodenal portal vein and malrotation: what causes the obstruction?

AIM AND METHOD: Preduodenal portal vein is a rare congenital abnormality, and occurs either as a single malformation, in association with other malformations or as part of "polysplenia" syndrome. Preduodenal portal vein has seldom been reported as a cause of intestinal obstruction, however corrective surgery is nearly always performed. We conducted a 25-year retrospective study in a single centre to investigate the cause of obstruction in patients with preduodenal portal vein. Furthermore, we reviewed the literature on preduodenal portal vein. RESULTS: Over a period of 25 years, preduodenal portal vein was diagnosed in five patients. The diagnosis was made during surgery performed because of symptoms of high intestinal obstruction. All five patients had intestinal malrotation as well and, in all patients, another cause for high intestinal obstruction than preduodenal portal vein was found. CONCLUSION: Preduodenal portal vein is mainly asymptomatic. It is often associated with other intestinal congenital abnormalities more likely to cause high intestinal obstruction. Therefore, the (paediatric) surgeon should always be alert for another associated cause of intestinal obstruction. Because of the potential for technical problems from preduodenal portal vein during surgery, it nevertheless should be on the surgeon's mind during surgery when the patient has high intestinal obstruction.