Radiology reporting of micturating cystourethrograms (MCUGs): What the paediatric urologists want to know.

BACKGROUND: Micturating cystourethrograms (MCUGs) are commonly requested to exclude vesicoureteric reflux (VUR) and bladder outlet obstruction (BOO). Useful additional information including timing and bladder volume at the start of reflux, urethral anomalies and post-void drainage can be obtained from the cystograms, but are not routinely reported by radiologists. OBJECTIVE: The study aim was to retrospectively review MCUG reports against a proposed reporting proforma, and then re-analyse the effect of the proforma on report quality. STUDY DESIGN: A retrospective analysis of paediatric MCUG reports was undertaken from two patient cohorts. Cohorts A (41 reports) and B (51 reports) comprised reports written before (2011-12) and following (2016-17) distribution of the standardised reporting proforma, respectively. Reports were assessed with respect to the parameters outlined on the standardised MCUG reporting proforma, including presence, grade and timing of VUR amongst others. Findings from both cohorts were compared and statistically analysed (p < 0.05 significant) to establish if the proforma influenced the content of reports. RESULTS: Statistically significant improvements were demonstrated in the reporting of: bladder outline normal/abnormal - reported in 92% after the proforma vs 56% before (p < 0.001); urethra normal/abnormal - 87% vs 68% (p = 0.033); contrast volume instilled - 84% vs 61% (p = 0.011); bladder emptying - 69% vs 17% (p < 0.001). In patients with VUR, reporting of VUR timing - 96% vs 33% (p < 0.001) and VUR grade - 91% vs 40% (p = 0.002) were also significantly improved. CONCLUSION: Implementation of a standardised MCUG reporting proforma produced substantial improvements in report quality and consistency, with statistically significant improvements noted in six of seven key features.

Complex presentation of intussusception in childhood.

The simultaneous occurrence of intussusception and volvulus in the paediatric age group is rare. We report the case of a volvulus of an ileoileal intussusception in an 8-year-old boy. This is the first time that computerised tomography (CT) images of a volvulus of an ileoileal intussusception have been published, and they clearly demonstrate both pathologies. This case highlights the use of CT in determining the nature of an abdominal mass and demonstrates how helpful it can be in diagnosing the cause of small bowel obstruction in children.

Childhood femoral hernia: a commonly misdiagnosed condition.

Femoral hernia is a rare surgical entity in the paediatric age group. The diagnosis is still a challenging issue. The aim of our study was to review our experience with femoral hernias in children during the past 21 years. We reviewed retrospectively the medical records of all patients who underwent femoral hernia repair in our institution between 1980 and 2000. Thirty-eight children (20 females, 18 males) with the median age of 5.5 years underwent 42 femoral hernia repairs over a 21-year period. Correct preoperative diagnosis was obtained in 20 (53%) cases. Of the 18 (47%) misdiagnosed cases, 11 were found to have a femoral hernia at negative exploration for a clinically diagnosis of inguinal hernia and 7 were found to have a femoral hernia 1 week to 18 months (mean 2.5 months) post repair of a presumed inguinal hernia. Median follow-up time was 12 years (range, 6 months-22 years). Recurrent femoral hernias occurred in five (13%) patients 6 months to 3 years (mean 13.5 months) after initial hernia repair. This study shows that femoral hernia is still a commonly misdiagnosed condition. A correct preoperative diagnosis will lead to appropriate surgical management, thus avoiding unnecessary morbidity and preventing unnecessary reoperations.

Pure alcohol injection of a congenital splenic cyst: a valid alternative?

Congenital splenic cysts are a rare entity. They are more frequent in children and young adults. They are true cysts, lined by epithelium with a typical trabeculation. Aspiration and injection with antibiotics (minocycline-tetracycline) or pure alcohol has been reported sparsely with variable results. The purpose of this case study is to report our experience with ultrasound (US)-guided aspiration and injection of a congenital splenic cyst, which, in our case, did not prove a viable alternative to surgical defenestration.

Alterations in smooth muscle contractile and cytoskeleton proteins and interstitial cells of Cajal in megacystis microcolon intestinal hypoperistalsis syndrome.

BACKGROUND/PURPOSE: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is characterized by decreased or absent peristalsis. Gastrointestinal motility depends on the enteric nervous system, smooth muscle cells (SMCs), and the interstitial cells of Cajal (ICCs). Contractile and cytoskeleton proteinase are important structural and functional components of SMCs. The aim of study was to examine the expression of contractile and cytoskeleton proteins in SMCs and distribution of ICCs in MMIHS bowel. METHODS: Full-thickness bowel specimens were obtained from 4 infants with MMIHS and 4 controls. Specimens were processed as whole-mount preparations and frozen and paraffin sections. Combined staining of NADPH-d histochemistry/c-kit immunohistochemistry, single and double immunohistochemistry using alpha-smooth muscle actin (alpha-SMA), calponin (CALP), caldesmon (CALD), desmin (DES), protein gene product 9.5 (PGP 9.5) and c-kit antibodies were performed and examined using light and confocal scanning microscopy. RESULTS: alpha-SMA, CALP, CALD, and DES immunoreactivity were reduced markedly in MMIHS bowel compared with controls. Combined NADPH/c-kit staining showed dense network of ICCs around myenteric plexus in MMIHS bowel. In contrast, the intramuscular ICCs either were absent or reduced in MMIHS bowel. CONCLUSIONS: Marked reduction of contractile and cytoskeleton proteins in SMCs combined with reduced expression of intramuscular ICCs in the gut may be responsible for the motility dysfunction in MMIHS.

Cervical thymic cysts.

Thymic cysts are considered uncommon lesions in the differential diagnosis of pediatric neck masses. They usually present in the 1st decade after the age of 2 years, possibly because the thymus attains its greatest development before puberty. They may be found anywhere along the normal descent route of the thymus gland from the mandible to the sternal notch; 50% extend into the mediastinum. Most patients are asymptomatic, although respiratory complications may occur. A review of the operative records in our hospital over the last 10 years revealed two cases of cervical thymic cysts (CTC) in a 5- and a 9-year-old boy. Both children presented with an atraumatic, painless, enlarging mass in the left side of the neck anterior to the sternocleidomastoid muscle. Neither boy had respiratory problems or swallowing difficulties. Ultrasound and computed tomography showed a lesion consistent with a tentative diagnosis of a branchial cyst in one boy and an extensive cystic hygroma in the other. Both lesions were approached through a transverse cervical incision and, although closely adherent to the internal jugular vein, carotid artery, and vagus nerve, were resected completely. There were no postoperative complications and so far there has been no recurrence. CTCs are uncommon benign lesions that should be considered in the evaluation of neck masses in children. Preoperative diagnosis is unusual and, at this time, there is no preoperative radiologic test that can accurately identify a neck mass as a CTC. Histologic investigation of the excised specimen showing thymic tissue remnants with pathognomonic Hassall's corpuscles and cholesterol clefts in the cyst wall is the only definitive diagnosis. Evolution is benign. Intact, complete surgical excision remains the treatment of choice.

Autoamputation of a congenital oropharyngeal hairy polyp.

Hairy polyps or dermoids are rare, benign congenital tumours of the oronasopharynx containing elements of both ectodermal and mesodermal origin. They occur most commonly in young infants and can cause respiratory distress or failure to thrive due to feeding difficulties. Computed tomography, magnetic resonance imaging, and barium video fluoroscopy can be useful in delineating the origin and extent of these lesions. Treatment usually consists of surgical removal. We describe a case of spontaneous resolution of symptoms in an 11-week-old baby due to autoamputation of the lesion.

Long-term outcome of the retained ureteral stump after lower pole heminephrectomy in duplex kidneys.

OBJECTIVES: Duplication of the ureter and renal pelvis is the most common upper urinary tract anomaly in childhood. The anatomical and functional divisions between upper and lower moieties of duplex kidney are extremely variable. The underlying pathological condition associated with a lower moiety is usually massive vesicoureteral reflux (VUR) to the lower collecting system and only rare obstruction. The non-functioning upper moiety is usually associated with obstructive ectopic ureter (with or without ureterocele). Most lower pole heminephrectomies are carried out for non-functioning lower moieties. In most cases, the lower defunctionalised segment of the ureter is left in situ. Complete ureterectomy is usually performed if presence of VUR into the lower end of the corresponding ureter is shown. There is little information on the long-term outcome of residual ureteral 'stumps'. The purpose of our study was to review the long-term outcome of retained ureteral stumps in children undergoing heminephrectomy for non-functioning lower pole moieties in duplex kidneys. MATERIALS AND METHODS: The medical records of 19 patients who underwent 20 lower pole heminephrectomies for a non-functioning lower pole moiety of a duplex kidney between January 1990 and December 2000 were reviewed retrospectively. Median age at heminephrectomy was 4.5 years (range: 1 month to 12 years). Indications for heminephrectomy in the 20 renal units was reflux nephropathy in 16 (80%) and obstructive nephropathy in 4 (20%). All corresponding ureters were taken down as low as possible and transfixed through the heminephrectomy incision. Median follow-up was 8.5 years (range: 1-11 years). RESULTS: Eight (40%) showed VUR into the stump after lower pole heminephrectomy. Two of these underwent subureteral endoscopic correction of VUR with polytetrafluoroethylene paste and resection of the stump was carried out in remaining two patients for recurrent urinary tract infections (UTI). Remaining four of the eight patients demonstrated spontaneous resolution of VUR during follow-up. CONCLUSIONS: Our data suggest that the vast majority of patients with residual ureteral stumps after lower pole heminephrectomy do not require stump resection at long-term follow-up.

Cholecystectomy versus cholecystolithotomy for cholelithiasis in childhood: long-term outcome.

BACKGROUND/PURPOSE: The presence of cholelithiasis is being reported with increased frequency in childhood. Little is known about the natural history of the disease, and only a few studies have been published regarding long-term results of treated patients. Controversy still exists regarding optimal treatment. Both cholecystectomy and cholecystolithotomy with gallbladder preservation have been recommended as the preferred operative intervention. The purpose of this study was to compare the long-term outcome of cholecystectomy versus cholecystolithotomy for symptomatic gallbladder disease in children. METHODS: The charts of all patients with symptomatic cholelithiasis treated in the Dublin Paediatric Hospitals during a 25-year period from 1974 till 1999 were reviewed. Data obtained included age, sex, age at presentation of symptoms, methods of diagnosis, indications for operative treatment, time interval between presentation of symptoms and surgery, surgical technique, performance of a preoperative or intraoperative cholangiogram, stone biochemistry, gallbladder histology, radiologic follow-up, the presence of recurrent or residual stones and symptoms, and the need for reoperation. Patient data were grouped according to method of surgery. All parameters were compared and evaluated. Follow-up was by way of telephone contact with all patients and completion of a questionnaire. RESULTS: There were 18 patients over a 25-year period. Eight patients underwent cholecystectomy, and 10 patients had a cholecystolithotomy. Median follow-up was 2 years in the cholecystectomy group and 5 years in the cholecystolithotomy group. All patients in the cholecystectomy group are asymptomatic and have no recurrent or residual stones on follow-up ultrasound scan. Thirty percent of the patients in the cholecystolithotomy group have recurrent right upper quadrant pain, and 30% show recurrent stones 9.5 months (range, 7 to 12 months) postoperatively. One patient underwent cholecystectomy 8.5 months postcholecystolithotomy. CONCLUSIONS: The symptomatic high stone recurrence rate postcholecystolithotomy seen in our series suggest that cholecystectomy is the preferred treatment in patients with symptomatic gallbladder disease.

Sacrococcygeal teratoma: results of a retrospective multicentric study in Belgium and Luxembourg.

Eighteen patients, operated upon for sacrococcygeal teratoma in 7 different centres in Belgium and Luxembourg between 1992 and 1996, were reviewed. From an epidemiological point of view, this series compares very well to others. Although excellent results were obtained, with all patients surviving, some imperfection in diagnosis, timing of delivery and of operation, and in operative technique was observed. Therefore, it is stated that for optimal treatment of sacrococcygeal teratoma to be achieved, these cases should be treated in just a very few centres of neonatal surgery.

Neonatal sigmoid volvulus: a complication of anal stenosis.

Sigmoid volvulus is an exceptionally rare cause of intestinal obstruction in neonates. Only 7 cases have been reported in the English and French literature. The authors report a recent case of sigmoid volvulus in a neonate secondary to anal stenosis and review the diagnosis and management of this serious condition. The authors believe that carefully performed radiologic reduction is the preferable alternative to surgical intervention.

Complex branchial fistula: a variant arch anomaly.

A 5-year-old boy presented with an infected left-sided branchial fistula. Despite antibiotic treatment and repeated excision of the fistula, purulent discharge from the wound persisted. Three-dimensional computed tomography (3D CT) reconstruction greatly facilitated the diagnosis and management of this case by showing the course of the fistulous tract. The complexity of the tract suggests that this represents a variant arch anomaly because it contains features of first, second, third, and fourth arch remnants.

Internal anal sphincter achalasia: outcome after internal sphincter myectomy.

BACKGROUND: Internal anal sphincter achalasia (IASA), also referred to as ultrashort segment Hirschsprung's disease (HD), is a clinical condition with presentation similar to HD, but with the presence of ganglion cells on rectal biopsy. The diagnosis of IASA is made on anorectal manometry, which shows the absence of rectosphincteric reflex on rectal balloon inflation. Altered intramuscular innervation has been reported in IASA. The purpose of this study was to review the outcome after internal sphincter myectomy in patients with IASA. METHODS: Fifteen consecutive patients (age range, 2 years to 12 years) with IASA underwent posterior internal sphincter myectomy. All patients presented with severe constipation with or without soiling. The diagnosis of IASA was made by anorectal manometry. HD was excluded in these cases by the presence of ganglion cells and normal acetylcholinesterase activity on suction rectal biopsies. Internal sphincter (IS) specimens were examined using immunohistochemistry for the general neuronal marker PGP 9.5 and synapsin 1 (a presynaptic marker) and using general histochemistry for NADPH-diaphorase. All patients underwent follow-up for periods from 2 years to 6 years. RESULTS: PGP 9.5, synapsin 1 and NADPH-diaphorase positive nerve fibers were either absent or markedly reduced in IASA specimens compared with controls, confirming previous reports of defective intramuscular innervation in IASA. At the time of follow-up, 7 patients have regular bowel motions and are not on any laxatives. Six patients have normal bowel habits but are on small doses of laxatives. One patient is able to stay clean with regular enema regimen. One patient required resection of dilated and redundant sigmoid colon and now has normal bowel habits with laxatives. CONCLUSION: The majority of patients with internal anal sphincter achalasia can be treated successfully by internal sphincter myectomy.

Cricopharyngeal achalasia: case reports and review of the literature.

Primary cricopharyngeal achalasia (a = absence, chalasia = relaxation) is a rare cause of swallowing disorders in newborns. Two cases are reported which were successfully treated by a myotomy of the cricopharyngeal muscle. A thorough history is essential in differential diagnosis as well as observation of the feeding infant. Presence of anatomical obstruction to swallowing and existence of neurological defects should be ruled out. Cineradiography with lateral views by an experienced radiologist is the best diagnostic technique. Esophageal manometry may provide information regarding other esophageal dyskinetic problems. However, these studies are difficult to perform in neonates and infants. Endoscopy may be helpful to exclude vocal cord paralysis or mechanical obstruction. Balloon dilatation has been reported as being successful in several reports; however no comparison of efficacy has been made in any series between dilatation of the upper esophagus and surgical myotomy which remains in our mind, the optimal treatment of cricopharyngeal achalasia.

Intraabdominal pyloromyotomy through the umbilical route: a technical improvement.

BACKGROUND/PURPOSE: Performing a pyloromyotomy through a supraumbilical skin fold incision will leave an almost invisible scar and therefore has definitive cosmetic advantages. This alternative approach may be related to technical difficulties in delivering a large pyloric tumor when compared with the conventional pyloromyotomy through a right upper quadrant incision. However, in situ (intraabdominal) myotomy can help overcome this inconvenience. METHODS: Of 122 cases of infantile hypertrophic pyloric stenosis operated on between January 1990 and August 1996, 29 underwent a pyloromyotomy performed intraabdominally through the umbilical route. The medical records of these babies were reviewed. RESULTS: Twenty-three boys and six girls (median age, 30 days; range, 17 to 70 days) underwent surgery. The median hospital stay was 2.5 days. There were two intraoperative technical complications (small mucosal perforation) and one postoperative wound complication (abcess formation) requiring local drainage. CONCLUSIONS: In situ pyloromyotomy through the umbilical route is an elegant alternative in cases of a large pyloric tumor.

Hemobilia: a rare complication of cholecystitis and cholecystolithiasis. Case report.

Hemobilia, although not uncommon, is usually inconsequential and most of the times not even diagnosed. It is known that hemobilia is a rare complication of cholecystitis and cholecystolithiasis. We report the case of a patient who presented with a life-threatening upper gastro-intestinal bleeding due to erosion of the cystic artery by cholecystolithiasis and cholecystitis.