Neonatal intestinal obstruction simulating meconium ileus in infants with long-segment intestinal aganglionosis: radiographic findings that prompt the need for rectal biopsy

Background: The initial clinical presentation and radiographic finding of microcolon in children with long-segment intestinal aganglionosis involving the entire colon, ileum and sometimes the jejunum can mimic meconium ileus. This makes the diagnosis difficult for the radiologist and surgeon. Objective: To document and describe the clinical and radiographic findings in children with long-segment intestinal aganglionosis who are initially thought to have meconium ileus. Materials and methods: We reviewed the cases of six neonates with long-segment intestinal aganglionosis presenting as meconium ileus at our institutions between 1978 and 2002. We examined the clinical presentation and the radiographic, surgical, and pathologic findings. In addition, 17 cases from the literature were identified and are included in the discussion. Results: A total of 23 cases were reviewed. Right lower quadrant intraluminal calcifications were noted on abdominal radiographs in all six neonates of our series and were described in 13 of the 17 neonates reported in the literature. Similarly, a microcolon was present in five of the six neonates of our series and in 14 of 16 historical neonates (one not reported). Conclusion: In a neonate with small-bowel obstruction and a microcolon, the presence of right lower quadrant intraluminal calcifications should raise the suspicion of long-segment intestinal aganglionosis even if the operative findings are typical of meconium ileus and a biopsy should be performed.     

Therapeutic strategies of meconium obstruction of the small bowel in very‐low‐birthweight neonates

Background: Meconium obstruction without cystic fibrosis in low‐birthweight neonates is a distinct clinical entity. We aimed to determine what therapeutic strategies work best in very‐low‐birthweight neonates with meconium obstruction of the small bowel under varied clinical conditions caused by the associated diseases of prematurity. Methods: Medical records of very‐low‐birthweight neonates with meconium obstruction of the small bowel treated from 1998 to 2008 were retrospectively reviewed. Pre‐ and postnatal data, treatments, and clinical outcomes were assessed. Results: Nine patients with perinatal complications were identified. Mean gestational age and birthweight were 26.9 weeks and 863 g, respectively. Abdominal distension developed from 1 to 7 days of life. Five patients were initially treated with Gastrografin enema, three of whom had successful outcomes. Two hemodynamically unstable patients failed to respond to Gastrografin treatment; they ultimately died of sepsis. The remaining four without Gastrografin treatment underwent enterostomy to resolve the obstructions with good results. Conclusions: Gastrografin and surgical treatments should be appropriately selected based on the underlying pathologies of meconium obstruction of the small bowel. Therapeutic Gastrografin enema is effective, safe and repeatable; however, it is not recommended for hemodynamically unstable patients. Surgical intervention is reserved for those who develop rapid abdominal distension that risks perforation.     

Contrast enema depiction of small-bowel volvulus in complicated neonatal bowel obstruction

About one-half of patients with meconium ileus (MI) present with a complication such as volvulus, atresia, meconium peritonitis or giant cystic meconium peritonitis. The treatment of these complications requires surgery. However, the preoperative diagnosis of complicated MI is difficult. We describe two neonates with complicated small-bowel obstruction, one with MI related to cystic fibrosis and the other not related to cystic fibrosis. In both, contrast enema depicted a spiral appearance of the distal small bowel, which at surgery proved to be the result of volvulus associated with antenatal bowel perforation. This appearance of the small bowel on contrast enema in this clinical setting has not been previously described. The recognition of this spiral appearance of the distal small bowel suggests the need for surgery.This paper was presented at the 41st Congress of the European Society of Paediatric Radiology, Heidelberg, Germany, June 2004     

Imaging of total colonic Hirschsprung disease

Background  Hirschsprung disease (HD) is a functional obstruction of the bowel caused by the absence of intrinsic enteric ganglion cells. The diagnosis of total colonic HD (TCHD) based on contrast enemas is difficult in newborns because radiological findings vary. Objective  To evaluate the radiographic and contrast enema findings in patients with pathologically proven TCHD. Materials and methods  From 1966 to 2007, 17 records from a total of 31 patients with TCHD were retrospectively evaluated for diameter and shape of the colon, diameter of the small bowel, bowel wall contour, ileal reflux, abdominal calcifications, pneumoperitoneum, filling defects, transitional zones and rectosigmoid index. Results  Three colonic patterns of TCHD were found: microcolon, question-mark-shape colon and normal caliber colon. Additional findings included spasmodic colon, ileal reflux, delayed evacuation and abdominal calcifications. Colonic transitional zones were found in eight patients with TCHD. Conclusion  The diagnosis of TCHD is difficult to establish by contrast enema studies. The length of the aganglionic small bowel and the age of the patient can influence the radiological findings in TCHD. The transitional zone and the rectosigmoid index can be false-positive in TCHD. The colon can appear normal. Consider TCHD if the contrast enema study is normal but the patient remains symptomatic and other causes of distal bowel obstruction have been excluded.     

T-tube ileostomy for intestinal perforation in extremely low birth weight neonates

To evaluate the results of use of T-tube ileostomy in selected cases of intestinal perforation in extremely low birth weight (ELBW) neonates. The records of 288 ELBW neonates treated at author’s institution, from 1998 to 2003 were retrospectively reviewed to identify neonates operated for intestinal perforation with T-tube placement. T-tube was inserted into the bowel through the site of perforation or proximally to the perforated gut via separate stab incision. T-tubes were used in five ELBW neonates (BW 600–900 g, gestational age 25–27 weeks) with intestinal perforation, in four of them at the time of primary surgery and in one neonate 8 days after primary anastomosis. All patients survived and there were no serious complications related to the T-tube insertion. Median duration of T-tube placement was 4 weeks (range 3–8 weeks), full enteral feeding after T-tube insertion was achieved in 4 weeks (range 1–6 weeks). All sites of T-tube insertion closed spontaneously. T-tube ileostomy is an effective and safe technique for treatment of selected cases of intestinal perforation in ELBW neonates. With respect to the hypoperistalsis of immature bowel, we recommend the use of T-tube in all cases of isolated intestinal perforation in ELWB neonates.     

Mesenteric hernia causing bowel obstruction in very low‐birthweight infants

Internal hernia through a mesenteric defect, called mesenteric hernia, is an uncommon cause of acute intestinal obstruction in newborns. Strangulated mesenteric hernia results in intestinal necrosis or perforation and progressive deterioration with fatal outcome, especially when it occurs in low‐birthweight infants. We report two very low‐birthweight (VLBW) infants, who presented with acute intestinal obstruction related to mesenteric defect. The initial diagnosis was meconium obstruction in those cases, which is a common cause of bowel obstruction occurring in VLBW infants. Correct diagnosis of mesenteric hernia was difficult in these cases because of rapid deterioration and non‐specific radiological findings. Awareness of the possibility of rare mesenteric hernia causing acute intestinal obstruction and surgical intervention in an appropriate timeframe are important for rescuing VLBW infants with such organic abnormalities.     

胎粪性肠梗阻临床诊治

目的探讨胎粪性肠梗阻的诊断和治疗方法。方法回顾性分析1989年～2004年收治的5例胎粪性肠梗阻的临床资料、手术方式、病理结果及预后。结果本组患儿多为足月产儿，术前诊断困难，术中发现本组患儿近端回肠扩张，结肠呈胎儿型，远端回肠内充满油灰样大便或直径1cm左右白色颗粒状、质地坚硬的胎粪栓。予泛影葡胺生理盐水稀释液行远端肠腔冲洗，取浆肌层标本送冰冻活检，分别予Bishop—Koop造瘘、回肠双腔造瘘。术后早期给予全胃肠外营养，平均术后6周关闭造瘘口。4例患儿恢复良好，1例自动出院。结论胎粪性肠梗阻须与全结肠无神经节细胞症相鉴别；Bishop-Koop造瘘可达到肠液丢失少，便于术后冲洗的良好效果。     

Management of meconium-related ileus in very low-birthweight infants

BACKGROUND: Although administration of a water-soluble contrast enema has been recognized to be effective for meconium-related ileus, there have been no definitive management guidelines for very low-birthweight infants. METHODS: Between 1998 and 2004, 10 infants without cystic fibrosis were treated for meconium-related ileus at Toyohashi Municipal Hospital. Their treatment and clinical course were reviewed retrospectively. RESULTS: The average gestational age and birthweight of the 10 infants was 27 weeks and 788 g, respectively. The average age at initiation of treatment with a water-soluble contrast enema was 6.8 days. Intestinal obstruction was relieved by the enema in eight of 10 patients, while one underwent laparotomy and one died without any improvement of obstruction. In both neonates for whom the enema failed, rectal examination and rectal irrigation had been performed for several days before the enema was administered at the age of 14 and 15 days, respectively. In contrast, the enema was administered at the age of 1-11 days in neonates for whom this treatment was successful. Obstruction was relieved if the contrast medium reached the distal ileum, but enemas without reflux into the distal ileum failed to improve the obstruction. Contrast medium passed through the ileocecal valve to reach the distal ileum in all procedures done under fluoroscopy, but the medium failed to reach the ileum in most of the procedures done without fluoroscopy. CONCLUSION: Although administration of water-soluble contrast enemas can be effective for meconium-related ileus, reflux into the terminal ileum is essential for bowel obstruction to improve, so it is desirable to perform the procedure under fluoroscopic guidance.     

Gastrografin enema in meconium ileus: the persistent approach

This report describes the value of repeated use of gastrografin enema in uncomplicated meconium ileus. Fourteen of 21 neonates had successful intestinal decompression (66.6%) — 5 after a single enema and 9 after repeated attempts (up to 7 times in 1 case). Offprint requests to: A. F. Azmy     

Megacystis microcolon intestinal hypoperistalsis syndrome

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare cause of intestinal obstruction mainly affecting female neonates. We present a case of a newborn female infant with a history of abdominal distension, bilious vomiting and decreased urine output. Barium enema showed a microcolon. Patient died soon after admission and the autopsy revealed a shortened bowel, a microcolon with abundant ganglion cells in the myenteric plexus, and an enlarged urinary bladder. An interesting finding in this case was the presence of enlarged nerve bundles containing several large ganglion cells on the lateral wall of the cervix. The salient clinical and autopsy findings in this case are presented.     

Neonatal gastrointestinal perforation in Japan: a nationwide survey

Purpose

The purpose of this study is to identify the current clinical features of neonatal gastrointestinal perforation in Japan.

Methods

A questionnaire about cases of neonatal gastrointestinal perforation treated in recent 5 years was sent to participating institutions of the Japanese Society of Pediatric Surgeons (JSPS).

Results

Five hundred and thirty-six neonates with gastrointestinal perforation were treated. They consisted of 42 patients with gastric rupture/perforation (GR), 33 patients with intestinal atresia/stenosis (IA), 3 patients with malrotation (ML), 118 patients with necrotizing enterocolitis (NEC), 160 patients with focal intestinal perforation (FIP), 46 patients with meconium-related ileus (MRI), 77 patients with meconium peritonitis (MP), and 57 patients with other conditions. The total mortality rate was 20.5 %. The mortality rates of the patients with GR, IA, ML, NEC, FIP, MRI, and MP were 9.5, 9.1, 0, 33.1, 20.6, 28.2, and 9.1 %, respectively. In 263 cases involving extremely low-birth-weight neonates (ELBW), 108 died (mortality rate 41.1 %). The mortality rates for ELBW with GR, NEC, FIP, MRI, MP, and other conditions were 27.3 % (3/11), 58.5 % (48/82), 21.6 % (24/111), 70.6 % (24/34), 57.1 % (4/7), and 27.8 % (5/18), respectively.

Conclusions

The mortality rates for ELBW decreased from 62.8 % in the previous survey to 41.1 % by the time of this survey.

     

Chronic intestinal pseudo-obstruction and delayed myenteric ganglion cell maturation]

BACKGROUND. Enteric nerve cells begin to mature during the last trimester of pregnancy and become mature only after birth. The degree of maturation seems to be related to bowel motility. CASE REPORT. A girl was born from a pregnancy complicated by hydramnios, and did not pass meconium before the 64th hr of life. Barium enema on day 4 showed a left microcolon with no distension of the transverse colon. Tests for cystic fibrosis were negative. On day 12, the patient presented with septicemia due to Pseudomonas maltophylia. Parenteral alimentation by central catheter was instituted. Surgical rectal biopsy showed that the number of ganglion cells was normal but the cells were immature. Progressive feeding was possible for the 3rd month of life. A second rectal biopsy at 3 1/2 months showed some remaining immature ganglion cells. CONCLUSION. Immature ganglion cells can account for neonatal functional intestinal obstruction, as has been established for the small left colon syndrome. The progressive loss of symptoms seems to be correlated with histological maturation of the neurological apparatus of the large bowel. Severe complications, such as occlusion, sepsis, nutritional disorders can occur during this long period of functional intestinal obstruction.     

Spontaneous gastrointestinal perforation in very-low-birth-weight infants – a rare complication in a neonatal intensive care unit

Over a 6-year period (1989–1995), gastrointestinal (GI) perforation was diagnosed in nine preterm infants (mean gestional age 27 weeks, mean birth weight 872 g). Three presented with necrotizing enterocolitis (NEC), two with indwelling-tube-induced perforation of the stomach, one with small-left- colon syndrome, and another with meconium ileus. Spontaneous intestinal perforation occurred in two similar very-low-birth-weight (VLBW) infants, in the distal ileum, on days 8 and 9 of life, respectively. The only clinical sign was extensive abdominal distension, and abdominal X-ray studies revealed free peritoneal air. All findings were distinct from those associated with NEC. Their further clinical course was complicated by reperforation on day 32 and 39, respectively. They subsequently recovered and presented without GI problems at the corrected ages of 4 and 2 months, respectively. In contrast to high mortality of 57% in the group with non-spontaneous intestinal perforations, spontaneous perforation seems to have a good prognosis even in VLBW infants if diagnosed and treated promptly. Accepted: 16 December 1996     

An unusual cause of meconium peritonitis in a foetus

Abstract:   Meconium peritonitis is a sterile chemical peritonitis resulting from intestinal perforation in-utero. We present a preterm male neonate weighing 1820 g in whom foetal meconium peritonitis was diagnosed by antenatal utrasonogram. Examination of the newborn in the immediate postnatal period revealed a firm lump of size 5 × 3 cm in the right lumbar and umbilical area. Rest of the abdomen was normal without any clinical evidence of intestinal obstruction. X-ray of abdomen done at 3 hours of life showed a cystic mass with calcified margin. Ultrasonography of abdomen ruled out any definite cause of intestinal obstruction and perforation. He was kept on conservative management. At 11 hours of life the neonate passed a small sticky white mucus plug per rectum followed by black colored meconium of normal consistency 2 hours later. The bowel habit remained regular thereafter. Screening for intrauterine infections and cystic fibrosis was negative. He is under regular follow-up and is now 6 months old with normal bowel habit. A lump of size 2 × 2 cm is still palpable and a small calcified mass is persistent in x-ray. Role of an intestinal mucus plug as the potential factor for intestinal obstruction and subsequent perforation has been highlighted in the present case.     

Meconium ileus secondary to cystic fibrosis The East London experience

Meconium ileus (MI) affects 15% of neonates with cystic fibrosis (CF). The authors reviewed the management and outcome of 51 neonates presenting to a single institution between 1976 and 1995 with MI secondary to CF. Clinical presentation included abdominal distension (96%), bilious vomiting (49%), and delayed passage of meconium (36%). A family history of CF was present in 4 cases (8%). Twenty-three neonates presented with MI and evidence of volvulus, atresia, or perforation (complicated MI). Of these, 16 underwent stoma formation, 1 appendicectomy, and 6 resection with primary anastomosis. Twenty-eight neonates presented with uncomplicated MI. Of these, 11 were managed non-operatively by Gastrografin enema (10) or enteral N-acetylcysteine (1). The remainder required stoma formation (15) or bowel resection with primary anastomosis (2). Early postoperative complications occurred in 2 neonates (4%). In this hospital the 1-year survival for this condition has increased from 49% (1953–1970) to 98% (1976–1995) irrespective of the surgical procedure performed or the presence of volvulus, atresia, or perforation. In our experience, bowel resection with primary anastomosis is as safe as stoma formation and is associated with a reduced length of initial hospital stay. Accepted: 23 October 1997     

Commentary

Five neonates with uncomplicated meconium ileus were successfully managed by laparotomy and T tube ileostomy. This method seems to offer advantages over other surgical techniques used in the management of uncomplicated meconium ileus that fails to respond to decompression by Gastrografin enema.     

Uncomplicated meconium ileus

Contemporary policy in meconium ileus (MI) management is to use different treatments in complicated and uncomplicated forms. The gastrografin enema is still used as a nonoperative form of treatment for uncomplicated MI, but is successful only in one-half of cases and may give rise to complications in one-fourth of cases. The authors report their experience in 29 neonates with MI.In the 12 complicated cases intestinal resection with primary anastomosis was carried out in 8 and resection with ileostomy in 4. In this group the overall mortality was 33%. In the 17 uncomplicated cases a gastrografin enema was attempted in 8. The success rate was 37.5% (3/8); in 3 cases enemas were unsuccessful and in 2 bowel perforations occurred, with 1 death. In all, 12 patients in this group were successfully treated by enterotomy and intraluminal irrigation without any complications. The authors now consider enterotomy and bowel irrigation to be the ideal surgical procedure for all uncomplicated cases of MI and not only for enema failures.     

Fetal meconium peritonitis without sequelae

Meconium peritonitis is a chemical peritonitis usually resulting from antenatal bowel rupture. Prenatal ultrasound findings include ascites, intraabdominal masses, bowel dilatation and the development of intraabdominal calcifications [1–5]. The most common bowel disorders which lead to meconium peritonitis in utero are those resulting in bowel obstruction and perforation, such as small bowel atresias, volvulus and meconium ileus [1–5]. Meconium ileus is associated with cystic fibrosis in most cases, although extraluminal abdominal calcifications are usually scarce in cases of cystic fibrosis [1, 6]. Postnatal outcome for infants with meconium peritonitis depends on the etiology for bowel rupture and underlying disease.     

Gastrointestinal complications in cystic fibrosis: meconium ileus equivalent

In contrast to bloatedness and steatorrhoea the meconium ileus equivalent is a less well known gastrointestinal complication in cystic fibrosis and thus less frequently correctly diagnosed. The term, first used by Jensen in 1962, notifies partial or complete obstruction due to increasing viscosity of gut contents. The occurrence of the meconium ileus equivalent increases with age (approx. 10%), recurrences are possible. Among 73 patients with cystic fibrosis 8 patients with ileus equivalents were observed, two of them had a recurrence Surgery was performed only in one case. In all other patients the intestinal obstruction was relieved by oral and enema administration of N-acetylcysteine.     

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.