Prenatal MR imaging of a meconium pseudocyst extending to the right subphrenic space with right lung compression

Meconium pseudocyst results from a loculated inflammation occurring in response to spillage of meconium into the peritoneal cavity after a bowel perforation. Certain cystic lesions, such as abscesses and dermoid and epidermoid cysts, are known to show reduced water diffusion on DWI. MRI has recently become a valuable adjunct to ultrasonography for fetal gastrointestinal anomalies. Complementary to ultrasonography, prenatal MRI can help further characterize the lesion and can clearly demonstrate the anatomical relationship between the lesion and adjacent organs. We report a case of meconium pseudocyst that was prenatally imaged with ultrasonography and MRI, postnatally complicated by pneumoperitoneum, and proved by postnatal surgery and histopathology. We emphasize the MRI of the pseudocyst, particularly T1-weighted and diffusion-weighted imaging.     

Calcified meconium balls in a newborn: an unusual case with imperforate anus,rectourinary fistula,colpocephaly, and agenesis of corpus callosum

Calcified intraluminal meconium is a rare finding in newborn infants. It is often associated with communication between the urinary and gastrointestinal tracts. Intra-abdominal calcifications are unusual radiographic findings in the newborn and can easily be misinterpreted as meconium peritonitis. We report on a newborn infant with anorectal malformation, meconium balls, intraluminal calcifications, colpocephaly, and agenesis of the corpus callosum, a rare association.     

Abnormal anatomy of the lumbosacral region imaged by magnetic resonance in children with anorectal malformations.

OBJECTIVE: To investigate the frequency of lumbosacral anomalies, the association with urogenital abnormalities, and the correlation with defaecation pattern by magnetic resonance imaging (MRI). METHODS: A prospective analysis was performed of routine MRI in patients with anorectal malformations. Between 1990 and 1994, MRI was performed in 43 such patients: 31 boys and 12 girls. Twenty four had a high anorectal malformation, 16 had a low anorectal malformation, and three had Currarino''s triad. MRI was performed before reconstruction in 26, and postoperatively in 17. Urogenital anomalies were found in 21. RESULTS: Abnormalities of the spinal cord and spine were found with MRI in 20 patients (46.5%); caudal regression syndrome in 10, tethered cord in two, a combination of both in three, and other spinal anomalies in five. These anomalies were found in 30% of the patients with low anorectal malformations, and in 50% with high anorectal malformations. In patients with urogenital malformations, MRI more often showed spinal anomalies (13/21, 62%) than in patients without (7/22, 32%). In high anorectal malformations, defaecation was more often a problem in patients with spinal anomalies (12/15, 80%) than in patients without (2/8, 25%). CONCLUSIONS: Spinal anomalies in the lumbosacral region were found with MRI in 46.5% of patients with anorectal malformations. Since presence of these anomalies seems to be related to clinical outcome, MRI should be performed routinely in all such patients.     

The anorectal sphincter after rectal pull-through surgery for anorectal anomalies: MRI evaluation

The puborectalis muscle sling was evaluated with MRI in 10 pediatric patients who had rectal pull-through surgery for anorectal anomalies. MRI (1.5T) demonstrated the puborectalis muscle equally or better than CT in 5 patients who had both CT and MRI. The amount of puborectalis muscle mass and the location of the pulled-through intestine in relation to the puborectalis sling was well shown with MRI. Our study suggests that MRI can be reliably substituted for CT in the majority of these patients, to assist in planning further surgery for relief of persistent postoperative fecal incontinence.     

Intraabdominal calcifications in the newborn: An unusual case with anorectal malformation and other anomalies

Intraabdominal calcifications are an unusual radiographic finding in the newborn and can easily be misinterpreted as meconium peritonitis. The authors refer to a patient with anorectal malformation (ARM), intraluminal calcifications and other anomalies. Careful differentiation of the type of intraabdominal calcifications can suggest the possibility of other initially unsuspected anomalies.     

Abnormal anatomy of the lumbosacral region imaged by magnetic resonance in children with anorectal malformations

OBJECTIVE: To investigate the frequency of lumbosacral anomalies, the association with urogenital abnormalities, and the correlation with defaecation pattern by magnetic resonance imaging (MRI). METHODS: A prospective analysis was performed of routine MRI in patients with anorectal malformations. Between 1990 and 1994, MRI was performed in 43 such patients: 31 boys and 12 girls. Twenty four had a high anorectal malformation, 16 had a low anorectal malformation, and three had Currarino's triad. MRI was performed before reconstruction in 26, and postoperatively in 17. Urogenital anomalies were found in 21. RESULTS: Abnormalities of the spinal cord and spine were found with MRI in 20 patients (46.5%); caudal regression syndrome in 10, tethered cord in two, a combination of both in three, and other spinal anomalies in five. These anomalies were found in 30% of the patients with low anorectal malformations, and in 50% with high anorectal malformations. In patients with urogenital malformations, MRI more often showed spinal anomalies (13/21, 62%) than in patients without (7/22, 32%). In high anorectal malformations, defaecation was more often a problem in patients with spinal anomalies (12/15, 80%) than in patients without (2/8, 25%). CONCLUSIONS: Spinal anomalies in the lumbosacral region were found with MRI in 46.5% of patients with anorectal malformations. Since presence of these anomalies seems to be related to clinical outcome, MRI should be performed routinely in all such patients.     

Low anorectal malformation associated with ‘ano-urethro-cutaneous’ fistula

Anorectal malformations are one of the common congenital anomalies encountered in the newborn period. The plethora of anomalies described makes it a complex subject for embryological explanations. Fistulous communications between the blind rectum and the urinary tract are common in boys with high/intermediate anomalies, but it is uncommon in children with low anorectal malformations, more so a double fistula. We are reporting a case of ‘ano-uerthro-cutaneous fistula’ associated with a low ano rectal malformation.     

Bowel function after surgery for anorectal malformations in patients with tethered spinal cord

Tethered spinal cord (TC) is an anomaly frequently recognized in association with anorectal malformations (ARM). However, the influence of TC on bowel function in children with ARM remains unknown. Furthermore, there are few studies that have assessed anorectal function in children with ARM and TC. The aim of this study was to evaluate anorectal function in ARM patients with TC using clinical assessment and anorectal manometry. Among 258 patients with ARM, this retrospective investigation included 35 patients who underwent spinal magnetic resonance imaging (MRI) after surgery for ARM. The patients were divided into two groups based on the presence or absence of TC, and bowel function was assessed by Kelly’s clinical score and anorectal manometry. Tethered cord was found in nine of the 35 patients (26%) with ARM. Of the ARM patients, TC was noted in four of 11 (36%) with high type anomalies, one of 8 (13%) with intermediate type anomalies, two of 14 (14%) with low type anomalies, and two of two patients (100%) with cloacal anomalies. Kelly’s clinical score did not significantly differ between the two groups. However, two of the nine patients with TC had poor bowel function (Kelly’s score; 2–0 points). On the contrary, patients without TC did not have poor bowel function. Anorectal manometry did not show a significant difference between patients with and without TC. However, the two patients with TC who had poor bowel function by Kelly’s score had low anal resting pressure, which was essential for achieving fecal continence. In conclusion, the present study showed that tethered cord was more frequently found in patients with more severe anorectal anomalies. Patients with TC were more likely to have poor bowel function, but this did not reach statistical significance. Presented at the 14th International Paediatric Colorectal Club, York, UK, 14–16 July 2007.     

An unusual cloacal anomaly—late recognition and operative correction

Persistent cloacal anomalies are usually recognised in newborn females. A 2-year-old presented with a cloacal lesion not previously documented. The precise diagnosis was delayed due to the fact that her external anatomy resembled a normal appearance. The urethro-vaginal and rectal canal opened through the anterior external anal sphincter complex. Chronic diarrhoea and genital excoriation ensued due to the mixing of the urine and faecal stream. The anatomy was defined by direct endoscopy, and radiological studies demonstrated a normal sacrum and bilateral renal tract anomalies. A preliminary diverting divided-limb sigmoid loop colostomy was followed by a posterior sagittal ano-rectoplasty with correction of the urethral, vaginal, and anorectal anomalies. The cosmetic appearance is very satisfactory. The urinary and faecal control in the child, whose unusual anomaly had been missed by many practitioners, both general and specialist, is very good. The renal anomalies now constitute the greatest threat to her longevity. Offprint requests to: J. F. Leditschke     

Imaging of congenital anomalies of the gastrointestinal tract

The radiological imaging plays a vital role in the evaluatin of patients with congenital anomalies of the gastrointestinal tract. The evaluation of these patients, most of which present early after birth, frequently requires the use of various imaging modalities for making the correct diagnosis and planning surgical correction. This article reviews the common congenital anomalies of the gastrointestinal tract including obstructive lesions, anomalies of rotation and fixation, anorectal anomalies, and intestinal duplications. The plain radiograph is often diagnostic in neonates with complete gastric of upper intestinal obstruction and further radiologic evaluation may be unnecessary. An upper gastrointestinal series should be performed in all patients with incomplete intestinal obstruction. Sonography is useful in the evaluation of many congenital anomalies affecting pediatric gastrointestinal tract especially hypertrophie pyloric stenosis, enteric duplication cysts, midgut malrotation, meconium ileus and meconium peritonitis. Moreover, CT and MRI has assumed a greater importance as these provide excellent anatomic details which may be necessary for correct diagnosis as well as treatment planning. This is particularly true in evaluation of congenital anomalies such as esophageal/enteric duplications, vascular rings and anorectal anomalies. It is important to be familiar with the role nad usefulness of the various imaging modalities so that these can be used judiciously to avoid unnecessary radiation exposure while minimizing the patient discomfort.     

Congenital funnel anus in children: associated anomalies,surgical management and outcome

Funnel anus (FA) is a rare and distinct type of anorectal malformation characterized by a skin-lined deep anal funnel, missing transitional epithelium and stenosis secondary to fibrotic internal sphincter. We aimed to characterize associated anomalies, surgical management and outcome of children with FA. The hospital records of 11 consecutive children (7 boys) treated for FA between 1992 and 2006 were screened. The collected data included the type of anorectal malformation, surgical management, associated anomalies, results of diagnostic investigations and outcome. Only one patient was free of any associated malformation. Six patients had a complete Currarino syndrome. Seven patients had a hemisacrum (scimitar) and tethered cord was present in two cases. Six patients underwent excision of a benign presacral teratoma. Anal stenosis associated with FA was managed by serial dilatations. Subsequent resection of the megarectosigmoid secondary to refractory constipation was performed on five occasions. Three patients underwent coloanal pull-through for Hirschsprung’s disease (HD). The level of aganglionosis was at the rectosigmoid junction in two cases and low in the rectum in one. One additional patient had hypoganglionosis. Of the three patients with HD two also had Down’s syndrome. After median follow-up of 6.5 (0.3–13.5) years four patients have normal bowel function and four suffer from soiling. Two patients with HD and Down’s syndrome and one patient with an undefined syndrome are fecally incontinent. Associated anomalies are common and diverse in children with FA. Pelvic MRI, sacral radiography, evaluation of the urinary tract and rectal biopsies are recommended as routine investigations in cases of FA. Surgical care of these patients is demanding and should be confined to dedicated centers.     

Utility of spinal MRI in children with anorectal malformation

Background  The association between spinal cord anomalies and imperforate anus is well recognized. Until now, the incidence of tethered cord has been assumed to be higher in patients with high-type imperforate anus. However, recent reports suggest that tethered cord is as common in patients with a low lesion as in those with a high lesion. Objective  To review the incidence of spinal cord anomalies in those with a low lesion and those with a high (including intermediate) anorectal malformation (ARM), and to determine the best diagnostic imaging strategy. Materials and methods  A group of 50 consecutive patients with postoperative ARM and in whom spinal MRI had been performed were identified retrospectively. We reviewed and compared the following factors between those with a high lesion and those with a low lesion: (1) clinical symptoms, (2) spinal cord anomalies, and (3) vertebral anomalies. Results  The incidence of spinal cord anomalies was no different between those with a high lesion and those with a low lesion, and spinal cord anomalies were present regardless of the presence of vertebral anomalies or symptoms. Conclusion  Owing to the high incidence of spinal cord anomalies in patients with imperforate anus, MRI is the best imaging tool for detecting such anomalies regardless of the level of the lesion.     

Inflammatory Cloacogenic Polyp in a Child: Part of the Spectrum of Solitary Rectal Ulcer Syndrome

A 15-year-old boy with Marshall-Smith syndrome presented with increased frequency and urgency of stooling, hematochezia, and rectal pain. A polypoid mass was found at the anorectal junction and excised. Microscopically, the lesion was covered by both squamous and columnar mucosa. It was villiform in configuration with focal ulceration and strands of smooth muscle in the lamina propria. These features are characteristic of an inflammatory cloacogenic polyp, a lesion not previously reported in the pediatric age group. Inflammatory cloacogenic polyp is related to solitary rectal ulcer syndrome and is most likely due to prolapse of the anorectal transition zone. Although rare in this age group, solitary rectal ulcer and its variants should be considered in the differential diagnosis of anorectal and rectal lesions in the pediatric patient.     

Evaluation of dysfunction following reconstruction of an anorectal anomaly

To evaluate the utility of anorectal manometry (ARM) and magnetic resonance imaging (MRI) with an endocoil in the assessment of dysfunction in children with repaired anorectal anomalies (ARA), 15 patients aged 1 to 15 years with repaired ARAs and chronic faecal incontinence or constipation were prospectively recruited. They underwent clinical assessment using a modified Wingfield score (MWS). ARM and MRI with an endocoil and conventional external coil were carried out. The results of ARM alone, MRI alone, and a combination of ARM and MRI were correlated with the MWS. Manometric internal anal sphincter (IAS) scores determined from sphincter length and activity correlated with MWS (r = 0.56, P = 0.02); manometric scores of rectal peristaltic activity did not. Overall manometric score (IAS and rectal scores combined) showed a correlation with MWS (r = 0.55, P = 0.02). Endoanal MRI sphincter scores did not correlate with MWS, but the presence of a megarectum on MRI did (r = 0.44, P = 0.05). Overall MRI score did not correlate with MWS. Minor neurosacral anomalies were shown on MRI in 3 children who had poor functional scores. Combined manometric and MRI scores showed a correlation with MWS (r = 0.58, P = 0.01). ARM and MRI are potentially useful in the assessment of dysfunction of children with repaired ARAs. Both modalities require refinement and further assessment in the context of directing management.     

US assessment of neonatal bowel (necrotizing enterocolitis excluded)

US plays a main role in evaluating neonatal gastrointestinal disorders. The authors describe the US appearance of normal neonatal bowel, midgut volvulus, small bowel atresia, meconium ileus, meconium peritonitis, different meconium plug syndromes, Hirschsprung disease, intestinal duplication cysts, some other abdominal cysts, some intraperitoneal infections, excluding necrotizing enterocolitis, and anorectal anomalies. The use of sonography in the imaging diagnostic strategies of these congenital and acquired anomalies is emphasized.     

Anal canal duplication in infants and children--a series of 6 cases.

The authors present a series of six anal canal duplications (ACD), duplications of the alimentary tract located along the posterior side of the anal canal, with a perineal opening just behind the anus. Five asymptomatic duplications were diagnosed before the age of one year, by simple perineal inspection. A twelve-year-old girl presented with perineal and anal pains and diarrhoea. Fistulography revealed a tubular structure in five cases and a cystic structure in one case, behind the normal anal canal, in one case communicating with it. A presacral sacrococcygeal teratoma was found in two children and in one case it was visualised by preoperative US in an infant with a lumbosacral myelomeningocele. Surgical excision was performed by a perineal approach in 5 cases, by a combined sacral and perineal approach in the last case, because of the associated teratoma. Non-invasive preoperative investigations, consisting of a pelvic X-ray, US examination, barium enema and fistulography, are sufficient in most cases; MRI is reserved for special indications. Surgical treatment restores a normal perineal aspect, without sequelae, and avoids complications like those described in other types of digestive duplications: infection, ulceration, bleeding, malignant changes during later adult life. Associated anomalies are frequently described in the literature, especially presacral tumours (16%) and anorectal malformations (21%); they can influence the management, the surgical approach and the functional prognosis.     

Hirschsprung's disease in the newborn

The records of all patients with Hirschsprung's disease diagnosed and treated at our institution between 1 July 1974 and 31 August 1985 were reviewed. Of these 99 patients, 35 (35%) presented and were diagnosed within the first 30 days of life and constitute the basis of the present study group. Twenty-two infants (63%) had standard rectosigmoid disease with a male-to-female ratio of 2.2:1. Only one infant was premature. The spectrum of presenting signs included abdominal distension in 19 (54%), failure to pass meconium within the first 48 h of life in 16 (46%), constipation in 12 (34%), and vomiting in 9 (26%). Intestinal perforation was a presenting sign in 2 patients (6%) and enterocolitis occurred preoperatively in 4 (12%). Evaluation was facilitated by diagnostic barium enema in 60% of the patients. In those infants able to undergo elective evaluation, the definitive diagnosis was made by suction rectal biopsy, which was accurate in all cases. In addition to the high proportion of patients with long-segment disease (13 patients, 37%), there was a significant incidence of associated congenital anomalies (26%), including Down's syndrome in 5 (14%). Thirty-three of the 35 patients have undergone definitive treatment using the endorectal pull-through procedure, performed at an average age of 12 months, with no mortality related to the operation. In addition to highlighting the high incidence of congenital anomalies, the large proportion of neonates with long-segment disease, and the reliability of the diagnostic barium enema, this subgroup of patients with Hirschsprung's disease emphasizes the special diagnostic and management considerations required in the newborn infant who presents with sepsis of unknown etiology, intestinal obstruction, or constipation. A high index of suspicion, liberal use of suction rectal biopsy, early leveling colostomy, and definitive treatment by endorectal pull-through are important in achieving 0% operative mortality and excellent functional results.     

先天性无肛畸形儿MR成像及其与排便功能关系研究

目的评估先天性肛门直肠畸形儿（anorectal malformations，ARM）尾端发育情况及横纹肌复合体（sphincter muscle complex，SMC）发育状态与排便功能的关系。方法39例ARM，男28例，女11例，应用不同序列体部相控阵列线圈或头部线圈MRI显示ARM直肠闭锁水平、骶尾椎、骶髓、泌尿生殖系的发育情况，并对SMC从多个层面进行定量分析，评估其发育状态与临床评分和肛门功能客观检查的关系。结果39例患儿中，MRI T1WI、T2WI扫描判断畸形水平的正确诊断率为100％，T2WI扫描瘘管检出率为96．9％。骶尾椎和骶髓的异常占41．0％，泌尿生殖系统畸形占20．5％。当耻骨直肠肌宽度的相对值PRWR〈0．18，外括约肌宽度的相对值EASWR〈0．15时，71．4％的ARM术后出现肛门失禁。当PRWR〉0．18，EASWR〉0．15时，91．3％的ARM术后排便功能好。结论MR检查能清楚地显示ARM类型、闭锁水平、SMC发育状态、脊柱脊髓及泌尿生殖系统存在的伴发畸形，从MR影像中找到了评价SMC的定量指标。     

Rectal or axillary temperature measurement: effect on plasma bilirubin and intestinal transit of meconium

Physiologic jaundice (5-6 mg/100 ml plasma bilirubin) is common and considered normal in newborn infants during the first week postbirth, but is considered abnormal in older infants. Early meconium passage correlates with lower plasma bilirubin concentrations. Rectal stimulation during rectal temperature measurement may aid passage of meconium containing potentially toxic, absorbable bilirubin. In this study, 100 clinically normal infants were assigned randomly to groups for rectal or axillary temperature measurement. The time of first meconium passage, time of first all-yellow stool, and total plasma bilirubin were compared between groups. Bilirubin was measured during metabolic screening at 48-80 h postbirth (mean 69 h in both groups). By this time 30 infants in the rectal group, but only 17 in the axillary group, had passed yellow stools (p = 0.011). The rectal group had lower mean (+/- 1 SD) bilirubin (5.5 mg/100 ml +/- 2.7 vs. 6.5 +/- 3.0, p = 0.042), and a trend toward earlier first meconium (5.9 h +/- 5.0 vs. 7.3 +/- 5.9, p = 0.096). For clinically normal infants in the first 48-80 h postbirth, these data suggest that rectal stimulation accompanying rectal temperature measurement enhances intestinal bilirubin excretion because of more rapid meconium passage.     

新生儿外科畸形的早期综合干预

目的通过产房外科等早期综合干预以提高新生儿外科畸形的临床诊治水平。方法对20例产前超声检查存在异常表现的胎儿进行B超和MRI随访观察，发现脐膨出6例，腹裂5例，消化道畸形6例，其他3例。18例行手术治疗，其中产房外科11例。术后多途径营养支持14例。采集脐带血20例。结果20例中治愈14例，因多发严重畸形和家属放弃治疗死亡6例。11例施行产房外科，其中腹裂5例，治愈4例；巨型脐膨出4例，治愈1例，术后31d意外死亡1例，放弃治疗死亡2例，普通型脐膨出2例Ⅰ期手术治愈。消化道畸形5例治愈，1例好转。脐带血成功回输12例。结论对新生儿外科畸形早期诊断和综合治疗，有助于避免严重并发症，降低病死率。产房外科的实施，能在最短时间内中断疾病对患儿的损害。产时脐带血采集和自体回输，提高了输血安全性。