Cloaca reconstruction: a new algorithm which considers the role of urethral length in determining surgical planning

Background

Cloacal malformations represent a uniquely complex challenge for surgeons. The surgical approach to date has been based on the common channel (CC) length with two patient groups considered: less than or greater than 3 cm, which we believe is an oversimplification. We reviewed 19 patients, referred after surgery done elsewhere. Eight had postoperative urinary complications, 3 had constant urinary leakage and had been left after surgery with a urethra < 1 cm, .5 with an original 3 to 5 cm common channel, who had undergone total urogenital mobilization (TUM), experienced peri-operative urethral loss needing a vesicostomy, and later, a Mitrofanoff. These patients together with a review of the cloacal and urological literature led us to design a new algorithm where urethral length is a key determinant for care.

Assessment of bowel control with anorectal manometry after surgery for anorectal malformation

Bowel function after surgery for anorectal malformation was assessed by Kelly's score in 101 children over 3 years of age. Seventy-two were examined using anorectal manometry. In the “good” group, resting pressure of the anal canal was as high as in the normal children, and the frequency of contraction waves in the anal canal was the same as in the normal children. Recto-anal reflex was recognized in about three fourths of the “good” group. On the other hand, in the “poor” group resting pressure was low, in only one were contraction waves evident and in none was there a recto-anal reflex. The findings of the anorectal manometry placed the “fair” group between the “good” and the “poor” groups. The clear contraction waves of the anal canal or the clear recto-anal reflex related to a resting pressure in the anal canal of over 20 cmH₂O. This high pressure in the anal canal is important for good anal continence, and depends on a precise pull-through of the rectum into the puborectalis muscle.     

Urethral length in female infants and its relevance in the repair of cloaca

Aim of the Study

The goals of urinary reconstruction in urogenital sinus and cloacal repair include: (1) positioning of the bladder neck above the urogenital diaphragm to maximize future urinary continence, and (2) creating a visible urethra that can be catheterized if needed. A recent algorithm in cloacal reconstruction proposed a urethral length of 1.5?cm as the key determinant in deciding whether to perform a total urogenital mobilization or a urogenital separation, the hypothesis being that a 1.5?cm length urethra is needed for the patient to remain dry. We wondered if the normal female urethral length correlated with this empiric technical determinant.

Laparoscopically assisted anorectovaginoplasty for selected types of female anorectal malformations

Purpose: The aim of this study was to describe laparoscopically assisted anorectovaginoplasty (LAARVP) for the repair of selected types of female anorectal malformation.Methods: Five cases (case 1, rectovaginal fistula with a high rectum; case 2, rectovestibular fistula with double vagina; case 3, rectovestibular fistula with absent vagina; case 4, anovestibular fistula with absent vagina; case 5, cloaca) were reviewed.Results: Patient 4 had undergone a posterior sagittal anorectoplasty without vaginoplasty at another hospital after misdiagnosis of simple anovestibular fistula. Mean age at LAARVP was 15.4 months. At LAARVP, the fistula was divided in cases 1 through 4 and dissected as low as possible in case 5. In cases 3 and 4, the distal fistula was used to create the neovagina. In cases 1 through 3, the proximal rectum was mobilized and brought through the pelvic floor sphincter muscles using Georgeson’s laparoscopically assisted colon pull-through technique through a minimal perineal incision. A posterior sagittal incision was required in cases 4 and 5. However, the initial laparoscopic approach was very helpful in both cases. Currently, all patients are well after a mean follow-up period of 32.2 months. Mean current age is 4.0 years. Patients 1 through 3 are continent, patient 4 is incontinent with soiling, and patient 5 is too young to be evaluated.Conclusions: LAARVP helps to achieve low dissection of the fistula, gives optimal view of the pelvic organs, provides accurate placement of the anorectal pull-through, and minimizes abdominal perineal scars.     

The transpubic approach for the correction of complex anorectal and urogenital malformations

Background

The transpubic approach has been used mainly to treat urethral injuries and prostate cancer. There are no reports describing this approach in anorectal malformations.

Methods

Forty-two patients who underwent a transpubic approach for their genitourinary/colorectal reconstruction were reviewed. Indications, complications, and follow-up were analyzed. A midline infraumbilical incision was used. The pubic cartilage was divided with needle cautery. Institutional review board approval was obtained (IRB# 2008-1317).

Results

The cases included complex malformations (16), covered exstrophy (15), long urogenital sinus with normal rectum (6), and reoperations in cloacas which had been left with persistent fistulae between vagina and urinary tract with normal rectum (5). Excellent exposure was achieved in all cases, allowing successful anatomical reconstruction. Functional results varied depending on the specific type of defect but were not expected to be good due to severe congenital or acquired anatomic defects. There were 3 complications related to the transpubic approach: pubic dehiscence, suspected osteomyelitis, and bleeding.

Conclusion

The transpubic approach should be considered for the repair of complex anorectal and urogenital malformations, especially when adequate exposure cannot be achieved with an abdominal, perineal, or posterior sagittal approach. Another ideal indication is in patients with a normal rectum who were born with a complex urogenital sinus or underwent a failed attempted repair but were left with problems requiring reoperation in a scarred and fibrotic pelvis.     

Laparoscopy and its use in the repair of anorectal malformations

Introduction

Laparoscopy has been used for the treatment of anorectal malformations (ARMs) in an attempt to be less invasive and with the hope that it would result in a better functional outcome. There remains a significant debate about whether these expectations have been fulfilled.

Methods

Seventeen patients with ARM for whom laparoscopy was used were retrospectively reviewed. Six were operated on primarily by the authors, and 11 cases were referred after a laparoscopic repair performed elsewhere. In addition, a literature review was performed looking for evidence of less invasiveness and improved functional results in patients operated on laparoscopically.

Results

The diagnosis was imperforate anus with a rectobladder neck fistula in our 6 cases with the fistula ligated laparoscopically in each case. In 1 patient, the malformation was repaired entirely using laparoscopic technique. The other 5 patients had a laparoscopically assisted repair because we had to open the abdomen to taper a dilated rectum in 2, mobilize a very high rectum in 2, and take down a distal colostomy stoma in 1. Eleven patients were referred with a variety of problems after a laparoscopic repair done elsewhere for rectal stricture (5), rectal prolapse (4), recurrent rectourethral fistula (3), rectal mislocation (3), failed attempted repair leading to fecal incontinence (1), and a posterior urethral diverticulum (1). Our literature review included 47 references (involving 323 patients) published between 1998 and 2010. All studies showed that laparoscopic repair of ARMs is feasible. The review, however, did not provide evidence of less invasiveness or improved functional results.

Conclusions

Laparoscopy for ARM is a less invasive procedure when compared with those operations that would have previously required a laparotomy (rectobladder neck fistula). In cases of rectoprostatic fistulae, the laparoscopic approach is feasible and avoids a lengthy posterior sagittal incision. There is no evidence that the laparoscopic approach is a less invasive procedure for other types of ARMs. In cases of rectobulbar fistula, congenital anal stenosis, perineal fistula, ARM without fistula, the evidence suggests that it may be lead to more complications. There is no evidence in the literature demonstrating better functional results in cases of ARM operated on laparoscopically.     

小儿肛门直肠畸形术后排便障碍的原因及治疗

目的探讨小儿肛门直肠畸形术后排便障碍的原因及治疗.方法 1985年1月～1998年12月共治疗肛门直肠畸形120例,对106例进行随访,其中男性78例,女性28例,年龄4～14岁,平均年龄10岁,随访率为80%.结果 38例高位肛门直肠畸形(36%),术后均有排便障碍(100%);32例中间位肛门直肠畸形(30%),术后15例有排便障碍(14%);36例低位肛门直肠畸形(34%),术后有6例排便障碍(6%).结论在高位肛门直肠畸形的病人中,术后均无正常排便,这可能与同时伴有脊髓神经及脊柱畸形有关.中间位及低位肛门直肠畸形术后有排便障碍与外科手术技术有关.腹腔镜手术技术应用于中高位肛门直肠畸形的治疗,使术后排便障碍大大减少,从而大大地提高了病人的生活质量.     

Transitions in care from pediatric to adult general surgery: Evaluating an unmet need for patients with anorectal malformation and Hirschsprung disease

Background

The provision of timely and comprehensive transition of care from pediatric to adult surgical providers for patients who have undergone childhood operations remains a challenge. Understanding the barriers to transition from a patient and family perspective may improve this process.

Influence of anesthesia on the results of intraoperative diagnostic electromyostimulation in patients with anorectal malformation

Background and Purpose

Stimulation electromyography (sEMG) is useful in identifying the sphincter muscle (M) in patients with anorectal malformations (ARMs). This study evaluates the effect of anesthetic agents and M relaxants on sEMG findings.

Materials and Methods

Seventeen infants (10 boys and 7 girls) with a mean age of 6.3 months and mean body weight of 6.7 kg were included in a prospective randomized study. Anesthesia was induced by sevoflurane and opioids, and an epidural catheter was inserted caudally. Stimulation electromyography of levator ani M using 14 mA current was used, and latency and amplitude of the evoked compound M action potential (CMAP) were recorded. Patients were randomized into 2 groups. Group A received a local anesthetic epidurally, and sEMG was performed. Administration of the M relaxant and measurement of M response followed. In group B drug administration, sEMG and response measurement were performed after administration of M relaxant.

Results

Baseline CMAP was recorded in all patients. Average latency was 4.1 milliseconds, and average amplitude was 0.43 mV. In group A, the average latency was 4.0 milliseconds, and average amplitude was 0.65 mV. After administration of the M relaxant, the CMAP disappeared. In group B, no CMAP was observed immediately after administration of the M relaxant.

Conclusion

Administration of the inhalational anesthetic, opioids, and local anesthetic did not influence the M response of M fibers in the levator ani M on sEMG and enables its localization during ARM reconstruction. Nondepolarizing M relaxation completely abolished the response. If M relaxant is necessary, cisatracurium is used. The most suitable method of anesthesia for ARM surgery appears to be inhalational anesthesia supplemented by opioids and epidural analgesia.     

The position of the anal dimple in newborns and infants with anorectal malformations and its correlation with the normal anal position

Background/purpose

The anal position index (API) defines the normal anal position as the ratio of fourchette–anal distance to fourchette–coccyx distance for females and the scrotum–anal distance to scrotum‐coccyx distance for males. In this study, measurement of the API in newborns and infants with anorectal malformations (ARM), using the center of the midline anal dimple (AD) to represent the center of the proposed neoanus, was performed to assess whether or not the AD was located in a significantly abnormal position as correlated with the normal anal position.

肛门直肠畸形术后排粪失禁患儿盆腔磁共振成像表现及其临床意义

目的通过肛门直肠畸形术后排粪失禁患儿的盆腔MRI表现,了解盆底肌的形态,直肠、肛管的形态和位置,以及脊髓和骶骨的发育,为分析排粪失禁的原因及制订治疗方案提供客观依据。方法回顾性分析2009年9月至2011年12月间山东大学第二医院收治的34例肛门直肠畸形术后排粪失禁患儿的临床和影像资料,其中男2l例,女13例,年龄3,14岁。应用1．5TMR扫描仪,常规行轴位、冠状位及矢状位扫描,观察肛门括约肌、耻骨直肠肌和提肛肌、直肠、肛管的形态,以及脊髓、骶骨的发育情况。结果MRI检查提示：肛门外括约肌发育不良18例,耻骨直肠肌发育不良23例,肛提肌发育不良27例;直肠位置异常6例,直肠扩张12例,肛直角增大11例;肛管周围脂肪组织5例;合并神经管闭合不全2例,Currarino综合征2例,骶骨发育不全11例,直肠尿道瘘2例。以上影像学结果均经临床最终证实。结论MRI可清楚显示肛门外括约肌、耻骨直肠肌和肛提肌的形态,以及直肠和肛管的形态和位置,同时还可显示脊髓和骶骨的发育情况,是评价肛门直肠畸形术后排粪失禁患儿非常有价值的检查方法。     

Diagnosis and management of a remnant of the original fistula (ROOF) in males following surgery for anorectal malformations

PurposeA complication of the surgical management of anorectal malformations (ARMs) is a retained remnant of the original fistula (ROOF) formerly called a posterior urethral diverticulum (PUD). A ROOF may have multiple presentations or may be incidentally discovered during the work-up of ARM after previous surgery. We sought to define the entity and the surgical indications for excision of a ROOF when found.MethodsWe performed a retrospective cohort study of all male patients who presented to our center following previous repair for ARM at another institution, who came for evaluation of problems with urinary and/or fecal continence, from 2014 to 2017. Charts were reviewed for symptoms, original type of malformation, preoperative imaging, treatment, and postoperative follow-up.ResultsOf 180 referred male patients, 16 had a ROOF. 14 underwent surgical repair to address this and for other redo indications, and 2 did not require intervention. 13 patients had an additional reason for a redo such as anal mislocation or rectal prolapse. Indications for ROOF excision were urinary symptoms (e.g. UTI, dribbling, passage of mucous via urethra, stone formation), to make a smoother posterior urethra for intermittent catheterization, or for prophylactic reasons. Patients were repaired at an average age of 4.2 years, using a PSARP only approach with excision of the ROOF for all except one patient who needed a laparotomy due to abdominal extension of the ROOF. No patient needed a colostomy. The original ARM repairs of the patients were PSARP (9), laparoscopic assisted (4) and abdominoperineal pullthrough (3). Preoperative evaluation included pelvic MRI, VCUG, and cystoscopy. The ROOF was visualized on 14 of 16 MRIs, 10 of 14 VCUGs, and 14 of 15 cystoscopies. Urinary symptoms associated with a ROOF and ease of catheterization were improved in all repaired cases.ConclusionPatients not doing well from a urinary or bowel standpoint post ARM pull-through need a complete evaluation which should include a check for a ROOF. Both modalities MRI and cystoscopy are needed as a ROOF can be missed on either alone. A VCUG was not reliable in identifying a ROOF.Excision is needed in patients to improve urinary symptoms associated with these lesions and to minimize the small but theoretical oncologic risk present in a ROOF.Level of evidenceLevel III.     

Laparoscopic-assisted anorectal pull-through for anorectal malformations: a systematic review and the need for standardization of outcome reporting

Introduction

Laparoscopic-assisted anorectal pull-through (LAARP) is becoming an increasingly common procedure to correct high and intermediate anorectal malformations (ARMs). The aim of this review was to evaluate worldwide experiences with LAARP with regard to indications, outcomes, and quality of reporting.

Method

A systematic review was conducted. The search was limited to studies reported in English and performed in humans. In addition to Medline and PubMed, a manual search of the Journal of Pediatric Surgery, Pediatric Surgery International, Surgical Endoscopy, and the Journal of Laparoendoscopic & Advanced Surgical Techniques published between June 2000 and April 2008 was conducted.

Results

Seventeen studies were included in the final analysis. Of the included studies, none were randomized, 2 were prospective in nature, and 4 compared outcomes of posterior sagittal anorectoplasty and LAARP. The studies included 124 patients (96 males, 28 females) with 80% reported as having high/intermediate malformations. All studies reported short-term outcomes. Reported outcomes included continence, rectal prolapse, the position of the rectum (7 studies using Kelly score), manometry (1 study), contrast enema (1 study), postanal endosonography (3 studies), and postoperative magnetic resonance imaging (3 studies). Outcomes varied widely between reports precluding a meta-analysis.

Conclusion

The number of studies dealing with LAARP is low. There is a need for both a standardization and improvement in the quality of reporting in LAARP research. This will ultimately allow for evidence-based surgical decision making.     

Concepts of combined endovascular and surgical treatment for dural arteriovenous fistulae: concepts derived from experience in treating three unusual lesions

Summary Background. We describe a treatment protocol for patients who have a dural arteriovenous fistulae (dAVF) not curable solely by an endovascular approach.Material and methods. Three patients suffering from neurological impairment due to a complex dAVM (intracranial haemorrhage 2, intracranial hypertension 1) were treated by a combination of arterial embolization treatment and subsequent surgery.Results. Marked reduction of flow was achieved by embolization of the main arterial feeders of the fistulae. Surgery eliminated the residual dAVFs completely and without permanent morbidity.Conclusion. It is necessary to eliminate a dural fistula completely, especially in a patient whose fistula had drainage or reflux into cortical veins, which have a high risk of intracranial haemorrhage and venous hypertension. We present an approach to treatment using transarterial embolization and early surgery which may offer a safe and effective way to achieve complete elimination of the lesion, avoiding increased risk to important venous drainage.     

Posterior decompression and in situ fusion for the treatment of degenerative scoliosis

Surgical Principles Asymmetric disc degeneration in the lumbar spine often creates a segmental instability that will result in progressive arthrosis of the facet joints and development of a lumbar scoliosis. Although the progression of the curve is slow, it may cause in the adult and elderly significant back pain and radicular pain [2, 3]. If conservative management fails, then surgical treatment should consist of a thorough decompression of the involved nerve roots and fusion of the involved vertebral segments. The technique described advocates an in situ fusion with no attempt at correction of the deformity and no use of instrumentation. This procedure is often required in elderly, osteopenic patients with systemic illnesses and therefore is designed to minimize intra- and postoperative morbidity.     

直肠后矢状纵切及心形吻合治疗先天性巨结肠

目的 提高先天性巨结肠的手术疗效。方法 回顾1992年8月至2002年8月应用直肠后矢状纵切、心形吻合技术治疗诊断明确的先天性巨结肠患儿106例，分析疗效及并发症防治。结果 106例患儿术后全部随访2个月～10年，并发小肠结肠炎5例，偶有污粪2例，便秘复发3例，无盆腔、腹腔感染及吻合口狭窄发生。结论 应用直肠后矢状纵切、心形吻合技术治疗先天性巨结肠，可提高手术疗效，减少并发症。     

Operation for delayed symptomatic brain oedema after treatment of an arteriovenous malformation by embolization and radiosurgery

Summary A patient with a Spetzler-Martin-grade-III AVM, initially embolized and then stereotactically irradiated, who – with a latency of several months – showed progressive neurological deterioration, is reported. Magnetic resonance imaging revealed enormous ipsilateral brain oedema, which did not respond to dexamethasone. Upon further neurological deterioration the former AVM nidus plus an adjacent rim of brain tissue were removed and the patient recovered considerably. It is concluded that embolization in conjunction with irradiation may impair the blood-brain-barrier with resulting long-term oedema in the surrounding brain, and that surgical treatment should be considered in selected cases.