Rectal mucosal dissection during transanal pull-through for Hirschsprung disease: the anorectal or the dentate line?

PURPOSE: Both the dentate line (DL) and anorectal line (ARL) are anatomic landmarks for rectal mucosal dissection during transanal pull-through for Hirschsprung disease. We compared outcome after rectal mucosal dissection commencing above the DL (DL group; n = 8) with outcome after rectal mucosal dissection commencing on the ARL (ARL group; n = 6) with normal babies (Cont group; n = 10). METHODS: Rectal mucosal dissection commenced on the ARL in the ARL group and at various levels (0-10 mm) above the DL in the DL group. Outcome was assessed prospectively for 6 months using a standard structured questionnaire. RESULTS: Subject demographics were not significantly different. Differences in frequency of motions between the ARL and Cont groups were not statistically significant after 3 months of age, but the DL group had significantly more motions than the other 2 groups after 4 months of age (P < .01). At 6 months of age, fecal staining was 17% in the ARL group, 63% in the DL group, and 0% in the Cont group. Anal shape was normal in the ARL and Cont groups, but 50% of the DL group had visible anal mucosa. CONCLUSION: Bowel function in the ARL group is similar to normal, and because the ARL is easily identifiable without the need for subjective interpretation, we recommend using the ARL as a landmark for rectal mucosal dissection during transanal pull-through for Hirschsprung disease.

Is intraoperative anal endosonography necessary during laparoscopy-assisted anorectoplasty for high/intermediate type imperforate anus?

PURPOSE: The aim of this study was to evaluate the relevance of anal endosonography (AES) during Georgeson laparoscopy-assisted anorectoplasty (GLA) for high/intermediate type imperforate anus (HIIA) using a continence evaluation questionnaire (CEQ) and postoperative AES (post-AES). METHODS: Fifteen HIIA patients who underwent GLA were enrolled in this prospective study. Six had intraoperative AES (op-AES) to confirm the accuracy of the positioning of the pull-through (PT) canal and 9 did not (no-AES). All subjects were reviewed prospectively by one surgeon and all had post-AES to measure the external sphincter (ES) and puborectalis (PR) at 3 and 9 o'clock to confirm that the PT colon was central. A CEQ (5 parameters: frequency of motions, severity of staining, severity of perianal erosions, anal shape, and requirement for medications; maximum score: 10) was administered to 9 subjects (4 from op-AES and 5 from no-AES) followed-up for over 3 years to assess continence. RESULTS: Mean age at GLA for both groups was not statistically different. There were no statistical differences between the two groups for thickness of the ES and PR on post-AES. Mean annual CEQ scores for the op-AES group 1, 2, 3, 4, and 5 years after GLA were 5.3, 6.3, 7.4, 8.4, and 8.5, respectively, while for the no-AES group were 5.9, 7.1, 8.1, 8.2, and 8.2, respectively; all differences were not statistically significant. CONCLUSIONS: Our results suggest that op-AES is not necessary during GLA because there is no difference in clinical and functional status that can be attributed to op-AES.

Anterior transposition of the third part of the duodenum for the treatment of superior mesenteric artery syndrome.

A 14-year-old adolescent girl with superior mesenteric artery syndrome was referred to us after failure of conservative management. Anterior transposition of the duodenum was performed and appears to be safe for permanently circumventing the duodenal obstruction seen in superior mesenteric artery syndrome, even in a pediatric population.

Midterm postoperative clinicoradiologic analysis of surgery for high/intermediate-type imperforate anus: prospective comparative study between laparoscopy-assisted and posterior sagittal anorectoplasty.

PURPOSE: The objective of this study is to analyze the outcome of surgery for high/intermediate-type imperforate anus using anal endosonography (AES), magnetic resonance imaging (MRI), and a continence evaluation questionnaire (CEQ). METHODS: In this study, 24 cases of high/intermediate-type imperforate anus were studied. Fifteen of 24 had Georgeson's laparoscopy-assisted anorectoplasty (GLA), and 9 of 24 had Pena's posterior sagittal anorectoplasty (PPA). All subjects had AES and MRI postoperatively. On AES, differences in thickness of the external sphincter (ES) and puborectalis (PR) at 3 and 9 o'clock were compared, and if pull-through colon was central, AES was 0. On MRI, differences in thickness were analyzed semiquantitatively and scored; if muscles were of even thickness, the score was 0; slight difference was 1, and marked difference was 2. A 5-parameter CEQ questionnaire (maximum score, 10) was administered to 16 of 24 subjects followed up for more than 3 years (9 GLA, 7 PPA). Surgical stress was assessed using mean febrile period, duration of raised white blood cell count, and peak C-reactive protein level. RESULTS: Mean age at surgery and mean postoperative period for both groups were not statistically different. There were no differences in mean muscle thickness for ES or PR on AES according to procedure (ES: GLA = 0.19 +/- 0.15 mm, PPA = 0.16 +/- 0.09 mm, P = .59; PR: GLA = 0.19 +/- 0.19 mm, PPA = 0.22 +/- 0.15 mm, P = .69). Magnetic resonance imaging scores were also not significantly different according to procedure (GLA: 0.77 +/- 0.83; PPA: 0.75 +/- 0.50, P = .97). When CEQ were compared annually, scores for GLA were generally higher throughout the study but only statistically significant at 3 and 4 years (P < .05). Differences in parameters of surgical stress were not significant. CONCLUSION: Although there were no differences in muscle thickness and parameters of surgical stress observed according to technique, GLA would appear to provide better outcome based on CEQ scores.

A comparative study of laparoscopy-assisted pull-through and open pull-through for Hirschsprung's disease with special reference to postoperative fecal continence.

AIM: The objective of this study is to compare laparoscopy-assisted pull-through (LPT) and open pull-through (OPT) for Hirschsprung's disease with special reference to postoperative fecal continence. METHODS: Thirteen OPT patients (1991-1996) were reviewed retrospectively, and 22 LPT (1997-2002) were reviewed prospectively. A continence evaluation questionnaire (CEQ, max score = 10) assessing frequency of motions, severity of staining, severity of perianal erosions, anal shape, and requirement for medications was used. Severity of staining was graded as none = 2, occasional = 1.5, often = 1, always = 0.5, and soiling = 0, and severity of staining less than or equal to 1 was defined as moderate to severe incontinence. Presence of fever (peak and duration), raised white cell count (>10,000/microL), and C-reactive protein (>0.3 mg/dL) were used to assess surgical stress. RESULTS: Pull-through was endorectal in all cases. Mean age at pull-through was not statistically different between the 2 groups. Annual CEQ scores for 7 years after LPT were 6.3, 6.9, 7.3, 7.7, 8.3, 8.9, and 9.0, and after OPT were 5.6, 6.4, 7.0, 7.5, 7.8, 8.3, and 8.4. Although CEQ scores were higher after LPT throughout, the difference was not statistically significant. The incidence of moderate to severe incontinence after 4 years was 54% (7/13) for OPT and 23% (5/22) for LPT, and after 6 years, it was 23% (3/13) for OPT and 0% for LPT. Duration/peak of raised C-reactive protein and duration of fever were significantly less for LPT (P < .01). CONCLUSION: Our results suggest that LPT is less invasive and may provide better postoperative bowel management compared with OPT.

Magnetic resonance urographic parameters for predicting the need for pyeloplasty in infants with prenatally diagnosed severe hydronephrosis.

PURPOSE: Treatment in infants with prenatally diagnosed, severe hydronephrosis remains controversial. We measured anatomical parameters using magnetic resonance urography to create a pelvic index ratio, which we correlated with outcome to assess its value. MATERIALS AND METHODS: We reviewed 35 cases of prenatally diagnosed severe hydronephrosis, including grade III in 19 and grade IV in 16. By 6 weeks after birth all patients had undergone repeat ultrasonography, diuretic renography and magnetic resonance urography. Magnetic resonance urography was used to measure the distance (D) from the bottom of the lowest renal calyx to the ureteropelvic junction and the total longitudinal length (L) of the caliceal system. The pelvic index ratio, defined as D/L, assesses the level at which the ureter inserts into the renal pelvis. RESULTS: All cases were initially managed conservatively. Pyeloplasty was required in 16 kidneys (mean patient age +/- SD 7.5 +/- 3.9 months) because of deterioration in renal function. All patients were well at a mean followup of 3.8 years. The remaining 19 cases continued to be managed conservatively. By a mean of 5.4 years spontaneous resolution of prenatally diagnosed severe hydronephrosis had occurred in 7 of 19 cases, while 12 were downgraded to grades I-II. The mean pelvic index ratio in surgical cases was 0.26 +/- 0.13 and in nonsurgical cases it was 0.12 +/- 0.11, indicating that ureters inserted significantly higher in surgical cases (p = 0.0012). Of 9 kidneys with a pelvic index ratio of greater than 0.3 pyeloplasty was required in 8, while 10 of 12 with a pelvic index ratio of less than 0.1 were managed conservatively. CONCLUSIONS: Pyeloplasty is likely to be required if the pelvic index ratio is greater than 0.3 and conservative management is likely to succeed if the ratio is less than 0.1. The pelvic index ratio can be calculated easily even in the neonatal period. It appears to be prognostic for pyeloplasty in cases of prenatally diagnosed, severe hydronephrosis.

Review of 86 patients with myelodysplasia and neurogenic bladder who underwent sigmoidocolocystoplasty and were followed more than 10 years.

PURPOSE: We reviewed the long-term outcome of sigmoidocolocystoplasty. MATERIALS AND METHODS: The records of 86 patients who underwent sigmoidocolocystoplasty with a followup of more than 10 years were reviewed, focusing on histopathology, renal function, vesicoureteral reflux, bladder stones and urinary continence. We developed a urinary continence evaluation score, calculated as the postoperative minus the preoperative urinary continence score, including 0-incontinence, 1-severe leakage, 2-moderate leakage, 3-mild leakage, 4-almost dry and 5-dry, for patient self-assessment of urinary continence. A score of 2 or more meant improvement and a score of less than 2 meant no improvement. RESULTS: Mean followup was 13.1 years (range 10 to 21). Histopathology showed inflammation in all cases, hyperplasia in 9 and metaplasia in 4. In 80 of 86 cases creatinine clearance was normal. Ureteral reimplantation had been performed before sigmoidocolocystoplasty in 9 cases (13 kidneys) for grades III to V vesicoureteral reflux, which recurred as grade I in 1 (1 kidney). Ureteral reimplantation was performed during sigmoidocolocystoplasty in 31 cases (58 kidneys) and grades I to II vesicoureteral recurred in 5 (6 kidneys). Of the remaining 44 sigmoidocolocystoplasty cases there were 2 (2 kidneys) of grade I vesicoureteral reflux. On (99m)technetium-dimercapto-succinic acid scintigraphy there was increased renal scarring in 8 cases. Bladder stones were identified in 18 of 86 cases during followup but they were absent at review. The urinary continence evaluation score showed improvement in 61 cases and no improvement in 25. CONCLUSIONS: Our results suggest that sigmoidocolocystoplasty is safe and effective in the long term.

Magnetic resonance imaging in lipoblastoma: can it be a diagnostic modality?

Magnetic resonance imaging (MRI) is generally an efficient tool for establishing the differential diagnosis of soft tissue tumours. We simultaneously encountered two patients with adipose tumours in the neck. Both had similar clinical features and MRI findings: lobular high intensity tumours on both T1-and T2-weighted images equivalent to subcutaneous fat with septal formation. The provisional diagnosis in both cases was lipoblastoma and both had complete excisions. Histopathological diagnoses, however, were different (lipoblastoma and fibrolipoma), indicating that while MRI is excellent for demonstrating the anatomical detail of soft tissue masses, it is not specific enough to be used alone for differentiating between adipose tumours. In this report, we assess the value of MRI and recommend a more appropriate and patient-friendly protocol for the evaluation for adipose tumours in children.

Mixed type I and II choledochal cyst: a new clinical subtype?

Traditionally, choledochal cyst (CC) is classified into five types according to Todani's classification. Of 356 cases of CC treated at our institute since 1965, we experienced four cases of mixed type I and II choledochal cyst (MTCC). The incidence of MTCC was 1.1%. All four patients had a fusiform common bile duct (CBD) with a diverticulum originating from the mid-portion of the CBD. The cystic duct drained into the right side of the diverticulum. All had pancreaticobiliary malunion (PBMU). All presented with recurrent abdominal pain. MTCC was suggested on ultrasonography or computerized tomography and confirmed by endoscopic retrograde cholangiopancreatography (three cases) or magnetic resonance cholangiopancreatography (one case). Age at surgery ranged from 3 to 12 years. All had hepaticoenterostomy, and total excision of the CC/diverticulum/gallbladder. There were no complications and all cases are currently well. In all cases, histopathology showed that the wall of the diverticulum was identical to the CBD. Although the incidence of MTCC is very low, pediatric surgeons should be aware of it as a new subtype of CC. Based on our experiences and a review of the literature, we suggest that its etiology does not affect treatment because the surgical management of choice is the same as for type I CC.

An extremely rare case of symptomatic right hepatic duct diverticulum located outside the liver.

Symptomatic hepatic duct diverticulum located outside the liver is extremely rare, with only one reported case. We encountered this anomaly and present our experience, focusing on surgical management. A 17-year-old-girl was referred to our institute because of recurrent right upper quadrant pain. At presentation, serum amylase, lipase, bilirubin, and transaminases were normal. White blood cell count and C-reactive protein were slightly raised. Ultrasonography and magnetic resonance cholangiopancreatography (MRCP) revealed a diverticulum-like lesion on the right side of the bile duct system appearing to overlap the gallbladder. The provisional diagnosis was type II choledochal cyst. Pancreaticobiliary malunion (PBMU) could not be confirmed on MRCP. The origin of the lesion could not be identified initially at laparotomy, but after repeated intraoperative cholangiography (IC) studies, the lesion was found to originate from a normal-sized right hepatic duct via a narrow duct. It was relatively easy to ligate the narrow duct and treat the lesion smoothly. IC also showed that the common bile duct was not dilated and that PBMU was absent. Histopathology showed the lesion to be a diverticulum of the bile duct epithelium. This is only the second report of a symptomatic hepatic duct diverticulum located outside the liver. Based on our experience, IC is particularly useful for the complete understanding of anomalous hepatobiliary duct anatomy and for planning surgical treatment.

Prospective analysis of primary modified Georgeson's laparoscopy-assisted endorectal pull-through for Hirschsprung's disease: short- to mid-term results.

The aim of this study was to analyze the short- to mid-term outcome of primary modified Georgeson's laparoscopy-assisted endorectal pull-through (PMGLEPT) for Hirschsprung's disease (HD). HD patients treated by PMGLEPT were evaluated prospectively by a single surgeon using a standard structured questionnaire to assess complications, incidence of enterocolitis, and evaluate continence (CE). CE involved scoring five parameters (frequency of motions, severity of staining/soiling, severity of perianal erosions, anal shape, and requirement for medications) on a 3-point scale (0, 1, and 2 for each parameter). Thus, scores for CE were: 10 = normal, 8-9 = good, 6-7 = fair, and 0-5 = poor. Our modifications include transanal rectal dissection starting below or on the dentate line, near total excision of the posterior rectal cuff, and intraoperative acetylcholinesterase staining to accurately identify normal colon. Patients with total colon aganglionosis or trisomy-21 were excluded, leaving 33 cases of PMGLEPT performed between 1997 and 2004. Mean operative age was 11.0 months. Follow-up ranged from 8 months to 7 years (mean 4.0 years). There were no intraoperative complications. Post-PMGLEP, bowel obstruction occurred in 1 subject who required middle colic division for pull-through (PT), and enterocolitis occurred in 3 (9.1%) of 33 patients. In 20 subjects aged over 3 years with a follow-up period of more than 12 months, final CE was normal in 5, good in 10, fair in 4, and poor in 1, despite staining/soiling being present in 12 (60%) of 20 subjects. None of the 33 had constipation. Our results suggest that PMGLEPT is safe with acceptable outcome in the short- to mid-term. However, careful long-term follow-up is mandatory as there appears to be a relatively high incidence of staining/soiling on short- to mid-term follow-up.

Serum soluble vascular cell adhesion molecule-1 (VCAM-1) concentrations in children with reflux nephropathy.

Systemic inflammatory disorders causing renal tissue damage do so by the adherence of polymorphonuclear leukocytes to endothelium, a process that is mediated by cell surface adhesion molecules. We determined the circulating levels of serum vascular cell adhesion molecule-1 (VCAM-1) in vesicoureteric reflux (VUR) patients and investigated the relationship between serum VCAM-1, grade of VUR, and secondary renal scarring. Serum levels of VCAM-1 were measured in 53 children aged between 3 months and 15 years with VUR (13 had grade III, 29 had grade IV, and 11 had grade V) and 25 controls using ELISA. Radionuclide scanning was used to assess renal scarring. Renal scarring was found in 29 of the 53 subjects. Serum VCAM-1 was significantly higher in subjects with high grades of VUR without renal scarring (grade IV: 715.9+/-121.0 ng/ml; grade V: 778.5+/-33.2ng/ml) compared with subjects with grade III VUR without renal scarring (609.8+/-64.3ng/ml, p<0.01). Serum VCAM-1 was also significantly higher in subjects with high grades of VUR with renal scarring (grade IV: 791.2+/-131.9ng/ml; grade V: 1171.8+/-235.6 ng/ml) compared with subjects with grade III VUR with renal scarring (687.3+/-163.4 ng/ml, p<0.001).

Giant, highly vascular sacrococcygeal teratoma: report of its excision using the ligasure vessel sealing system.

Reports about giant, highly vascular sacrococcygeal teratoma (GHV-SCT) are uncommon. A boy with a prenatally diagnosed GHV-SCT was born at 34 weeks' gestation weighing 3,716 g. At birth, the GHV-SCT was 20 x 17 x 14 cm. After initial devascularization using an aortic snare, excision of the GHV-SCT was started using monopolar diathermy, but hemorrhage from cutaneous collateral vessels was difficult to control, and individual ligation of vessels was extremely time consuming. The authors then used the LigaSure (LS) vessel sealing system (Valleylab, Boulder, CO) to control intraoperative hemorrhage. Hemostasis was achieved easily with LS, without any need for vessel ligation; the operative field was dry and the efficiency of excision enhanced. Total operating time was 255 minutes, but excision of the GHV-SCT itself took only 16 minutes. The GHV-SCT weighed 1,208 g or 325.1 g/kg body weight. Total blood loss was 77 mL or 20.7 mL/kg body weight, which was minimal compared with other cases in the literature. At the last follow-up examination, 7 months after surgery, he remains well with no evidence of recurrence. LS was extremely useful for controlling intraoperative hemorrhage in this case, and we strongly recommend its selective use for excising highly vascular SCT.

Associated urologic anomalies in children with solitary kidney.

PURPOSE: The aim of this study was to investigate the incidence, nature, surgical treatment, and outcome of associated urologic anomalies (AUA) in children with anatomic or functional solitary kidney (SK). METHODS: In this study, SK was defined as the kidney that is present in cases of unilateral renal agenesis (RA), or the kidney that is contralateral to a kidney that is nonfunctional secondary to either noncystic dysplastic kidney disease (NCDK), or multicystic dysplastic kidney disease (MCDK). Fifty-seven consecutive patients with SK (17 owing to RA, 10 owing to NCDK, 30 owing to MCDK) were reviewed. RESULTS: A total of 23 of 57 (40%) had AUA in the SK (11 of 17 [65%] of RA cases; 7 of 10 [70%] of NCDK cases; and 5 of 30 [17%] of MCDK cases). The incidence of AUA in MCDK was significantly lower than that in RA or NCDK (p <.01: Mann-Whitney U test). Surgery was performed on 20 of 23 (87%) for vesicoureteric reflux (VUR) in 13, pelviureteric junction stenosis in 3, and ureterovesical junction stenosis in 4. Surgery was not performed in 3 of 16 with VUR because the VUR that was present was of low grade. AUA-related symptoms such as urinary tract infection and abdominal mass were seen in 15 of 23; the remaining 8 were asymptomatic. Mean age at surgery was 4.8 years. There were no operative or postoperative complications, and all cases are well without impaired renal function or hypertension after a mean follow-up period of 5.3 years. CONCLUSIONS: In patients with SK, 40% had AUA in the renal collecting system of the SK. All children with SK should undergo a screening voiding cystourethrography (VCUG) even in the absence of hydronephrosis or UTI, and early recognition and treatment are imperative to decrease the long-term risk for renal damage.