Urinary continence after cloacal repair is difficult to achieve and renal outcome in patients with cloacal malformations has been scarcely reported. As a result, we reviewed our experience with cloacal malformations to determine the status of continence and the long-term renal function in these children.
A retrospective chart review from 1990 to 2003 identified 12 patients with cloacal malformation (1 posterior, 4 complex and 7 classical) who underwent surgical reconstruction. The confluence was defined as high (common channel ≥ 3 cm) and low (< 3 cm) by cystovaginoscopy. Renal ultrasound, voiding cystouretrogram, renal scan and sacral radiograph were performed in all children. Most patients underwent 1-stage abdominoperineal pull-through, applying the principle of total urogenital sinus mobilization. We collected data regarding hydronephrosis, vesicoureteral reflux and split differential renal function. Renal outcome was evaluated based on glomerular filtration rate and age-adjusted serum creatinine values (μmol/L). Urinary continence was defined as a dry interval > 4 hours.
Patients' mean age at surgery was 20 months (range 7–29 mo). Of the 12 children who underwent cloacal repair, 7 (58.3%) had a common channel ≥ 3cm. Renal anomalies were identified in 3 of 12 (25%) girls: there were 2 solitary kidneys and 1 pelvic kidney. Lumbar–sacral radiography demonstrated bony abnormalities in 11 of the 12 (91.6%) cases: hemivertebra in 3 cases, sacral agenesis in 4 cases, hypoplastic sacrum in 3 cases and bifid sacrum in 1 case. Total urogenital sinus mobilization through an abdominoperineal approach in a single stage was performed in 8 girls. Follow-up ranged from 4 to 14 years (mean 8.5 yr). Eight (66.6%) children had dry intervals > 4 hours, 5 (62.5%) of them were on clean intermittent catheterization through a Mitrofanoff channel and 1 (12.5%) was through the urethra. The remaining 2 (25%) patients were voiding spontaneously. Three (33.3%) patients were totally incontinent, and 1 (8.3%) patient was awaiting reconstruction. The mean measured glomerular filtration rate was 93.5 mL/min/1.73m2 (range 34–152 mL/min/1.73m2). Four (57.1%) of 7 patients who had a common channel ≥ 3 cm ended up needing augmentation cystoplasty, compared with none of the patients with a common channel < 3 cm (57.1% v. 0%, p = 0.038).
Urinary continence can be achieved in most patients with cloacal malformation at the expense of major reconstructive surgery and despite the presence of associated urological abnormalities. However, these children harbour an important risk for renal impairment later in life and should be closely monitored.
Persistent cloaca is one of the most severe types of anorectal malformation. Appropriate initial drainage is difficult due to their various malformations and hydrocolpos or dilated urinary bladder. Corrective surgery also differs among individual patients. We describe our experiences with the surgical management of children with persistent cloaca.
We retrospectively reviewed 16 children diagnosed with persistent cloaca at Asan Medical Center.
Sixteen patients were managed in their neonatal period. Twelve patients had enlarged bladder or vagina at birth. Three patients, who did not undergo cystostomy or vaginostomy at first operation, had earlier complications after surgery or required drainage tube insertion. One patient who did not undergo hydrocolpos drainage died of sepsis and complications. Nine patients underwent corrective surgery; posterior sagittal anorectovaginourethroplasty using the Pena method. Three patients required additional operations due to complications after surgery.
Patients found to have anatomical malformations before colostomy, as well as hydrocolpos and bladder enlargement, require a vaginostomy with or without a cystostomy to reduce complications. Follow-up is required in patients with hydrocolpos and bladder enlargement to determine whether vaginal drainage improves dilated bladder. Continuous long-term follow-up examination is required to determine the long-term results of corrective surgery.
Persistent cloaca; Anorectal malformation; Hydrocolpos; Posterior sagittal anorectovaginourethroplasty
Robotic-assisted radical cystectomy (RARC) is an emerging minimally invasive alternative for the treatment of invasive bladder cancer. The V-loc (Covidien, Mansfield, MA) suture is a unidirectional barbed suture that provides even distribution of tension. We determined the rate of urinary leak at the urethro-intestinal anastomosis following orthotopic neobladder construction performed with V-loc suture.
We retrospectively reviewed charts on all patients that underwent RARC with orthotopic neobladder urinary diversion performed with a V-loc suture from February 2010 to February 2012. The urethro-intestinal anastomosis was evaluated for urinary leak by cystogram at 3 to 4 weeks, postoperatively.
In total, 11 patients were available for analysis. The mean patient age was 57.2 years (range: 47–71). The average clinical follow-up was 8 months (range: 4–15). On surgical pathology, 8 (73%) patients had pT2 or less disease, 3 (27%) had pT3/T4 disease, and 1 (9%) had N+ disease. The mean intraoperative blood loss was 315 mL (range: 150–600) and the average operative time was 496 minutes (range: 485–519). No patient (0%) demonstrated a urinary leak at the urethro-intestinal anastomosis on postoperative cystogram. Eight patients (73%) were continent by 4 months, postoperatively.
Orthotopic neobladder urethro-intestinal anastomosis can be performed effectively and safely with V-loc suture with an acceptably low urinary leak rate.
A rare case of squamous cell carcinoma of bladder occurring in a 36-year-old female with persistent cloacal anomaly who presented with frequency, urgency, dysuria, and recurrent urinary tract infection is reported. Contrast Enhanced Computed Tomography with three dimensional reconstruction showed presence of bladder tumor and persistent cloaca. She underwent pelvic exenteration and wet colostomy. Histopathologic findings revealed locally advanced moderately differentiated squamous cell carcinoma.
Bladder tumor; cloacal anomaly; urinary tract infection
Colostomy site carcinomas are rare with only eight cases reported in the world literature. Various etiological factors like adenoma-cancer sequence, bile acids, recurrent and persistent physical damage at the colostomy site by faecal matter due to associated stomal stenosis have been considered responsible. Two such cases are being reported and in both cases there was no evidence of any local recurrence in the pelvis or liver and distant metastasis. Both patients had received adjuvant chemotherapy following surgery.
First case was a 30-year-old male that had reported with large bowel obstruction due to an obstructing ulcero-proliferative growth (poorly differentiated adenocarcinoma) at the colostomy site after abdomino-perineal resection, performed for low rectal cancer six years previously. Wide local excision with microscopically free margins was performed with a satisfactory outcome. Four years later he presented with massive malignant ascites, cachexia and multiple liver metastasis and succumbed to his disease.
Second case was a 47-year-old male that presented with acute large bowel obstruction due to an annular growth (well differentiated adenocarcinoma) in the upper rectum. He was managed by Hartmann's operation and the sigmoid colostomy was closed six months later. Five years following closure of colostomy, he presented with two parietal masses at the previous colostomy site scar, which, on fine needle aspiration cytology were found to be well-differentiated adenocarcinomas of colorectal type. Surgery in the form of wide local resection with free margins was performed. He presented again after five years with recurrence along the previous surgery scar and an incisional hernia and was managed by wide local excision along with hernioplasty. Follow-up of nine years following first surgery is satisfactory.
Colostomy site/scar recurrence of rectal carcinoma is rare and could be due to various etiological factors, although the exact causative mechanism is not known. Surgery with microscopically free margins is recommended in the absence of metastatic disease. Stenosis of the stoma is considered as one of the most important contributory factors and should be followed carefully.
Report of seven children with Y-type urethral duplication (YUD).
Materials and Methods:
(A) Four staged operations were performed in patients having extensive perineal dissection (for rectourinary fistula separation and anterior mobilization of ventral urethra (VU)), tension rectocutaneous anastomosis, and children who were not toilet trained). These stages are (1) diversion sigmoid colostomy with anterior mobilization of VU as perineal urethrostomy via anterior sagittal approach; (2) Orthotopic urethral (OU) reconstruction; (3) anastomosis of OU and perineal urethra; (4) colostomy closure with management of complications. (B) The patients having VU onto the perineum underwent single stage urethral reconstruction.
The VU was urethrorectal/urethroanal in five and urethroperineal in two. Low anorectal malformation and upper urinary tract anomalies were present in 57.1% (4/7) and 14.7% (1/7) patients, respectively. Buccal mucosa free graft, transverse inner preputial flap, and perineal skin were tubularized for OU reconstruction. Mean age at 1st , 2nd , 3rd , and 4th surgery was 5 ± 0.78, 28 ± 0.78, 36 ± 0.78, 49 ± 0.78 months respectively. Three patients needed surgery for complications (urethrocutaneous fistula in two and urethral diverticulum in one) in a mean 3.12 ± 0.34 years of follow-up. Final uroflowmetry and fecal continence were good in all patients.
The YUD is a difficult entity to manage. Although, staged procedure appears to be time consuming, but good and promising results can be achieved by staging the procedure.
Anourinary fistula; perineal urethrostomy; rectourinary fistula; urethral duplication; Y-type urethral duplication
Stercoral perforation of the colon has rarely been reported. Only 3 cases of stercoral perforation of the colon proximal to an end colostomy have been reported. We present two cases of stercoral perforation of the colon in end colostomy patients.
PRESENTATION OF CASE
A 70-year-old man who had undergone abdomino-perineal excision for anal cancer was referred for left lower quadrant pain and fever. Stercoral perforation was discovered along the distal descending colon, proximal to the end sigmoid colostomy. The patient underwent segmental resection of the colon and revision of the stoma and was discharged on postoperative day 32. A 71-year-old woman who had undergone abdomino-perineal excision for distal rectal cancer with preoperative chemoradiation presented fever with 2 days of low abdominal pain. The patient had sacral bone and lung metastases from rectal cancer and suffered from chronic constipation. Stercoral perforation was found around the sigmoid colon, just proximal to the end sigmoid colostomy. The patient underwent simple repair of the perforated colon through the parastomal incision. On postoperative day 8, leakage occurred at the repair site. Segmental resection of the colon and revision of the stoma were performed. She was discharged 44 days after the initial surgery.
Segmental resection of the perforated colon, rather than simple repair, appears to improve postoperative outcomes.
As the number of cancer survivors increases, appropriate management of constipation is important to prevent stercoral perforation during follow-up.
Stercoral perforation; Colon; Colostomy
The exstrophy-epispadias complex is a rare congenital malformation of the genitourinary system, abdominal wall muscles, and pelvic structures. Modern surgical repairs focus on reconstruction of the bladder and its adjacent structures, with the goal of achieving urinary continence, a satisfactory cosmetic result, and a high quality of life. Complex surgery in neonates and young children, as well as a prolonged postoperative course require close collaboration between surgeons, anesthesiologists, intensivists, pediatricians, and an experienced nursing staff. This article will review the spectrum of bladder exstrophy anomalies, the surgical repair, and the perioperative interdisciplinary management.
bladder exstrophy; infant; newborn; surgery; anesthesia
We report an incarcerated internal hernia in a huge irreducible parastomal hernia-"hernia within hernia." A 70-year-old obese woman with diabetes who underwent an abdomino-perineal resection 20 years ago was admitted to our hospital with 20 years history of a huge irreducible bulge, 25 cm in diameter. An internal hernia due to an adhesive band extending from the sac wall to proximal colon was found in the parastomal hernia sac during an emergency laparotomy. We cut off the distal colon and relocated the colostomy stoma. The patient was discharged uneventfully 2 weeks after the surgery and was readmitted to have a further laparoscopic hernia repair 8 months later. Unfortunately, an unrecognized enterotomy occurred during the secondary surgery that led to an additional laparotomy during which the mesh was not contaminated by the bowel contents and was kept in place. At 22-month follow-up, there were no evidences of recurrence.
Ventral hernia; Incarceration; Intestinal obstruction; Herniorrhaphy
The results of the new reconstruction of the rectum after Abdominoperineal Resection (APR) with ‘S’-trap arrangement of the colon and continent perineal colostomy controlled by adynamic bilateral graciloplasty are evaluated. The fully stretched gracilis muscles were utilized for occlusion of the lumen of the colon. METHODS and MATERIALS: Between April 1993 and December 2006, selected 42 patients (30 males and 12 females) with median age of 43.5 years (25–64 years) were treated by a one-stage procedure without colonic diversion. All patients were suffering from adenocarcinoma of the lower third of the rectum. The abdominoperineal resection was carried out in all cases. A 25-cm-long vascular segment of the colon was installed in the sacral curve and ‘S’-shaped trap was developed with fixed colonic curves at the coccyx and to the left ischial rami of the pubic bone. The left gracilis was wrapped around just distal to the fixed curve of the colon to the left ischial rami and right one around the perineal colostomy with different grades of tightness. Continence was satisfactory in 34 patients; they could hold more than 1,000 ml of saline. Occasional soiling was reported in the night in 8 patients. All patients have achieved near-natural continence. The new rectal reconstruction with ‘S’ arrangement of colon and continent perineal colostomy achieved near-natural continence in 66% of cases.
Rectal reconstruction; APR; Continent perineal colostomy; ‘S’ arrangement of the colon; Modified transposition of the gracilis; Bilateral adynamic graciloplasty
With the advance of prenatal imaging, more often pediatric surgeons are called for prenatal counseling in suspected cases of cloaca or cloacal exstrophy. This presents new challenges for pediatric surgeons since no specific guidelines have been established so far. The purpose of this review is to analyze our experience in prenatally diagnosed cloaca or cloacal exstrophy and to provide some guidelines for prenatal counseling of these complex congenital anomalies.
A retrospective review of the medical charts of patients with prenatally diagnosed cloaca and cloacal exstrophy who received postnatal care in our institution between July 2005 and March 2012 was performed. Representative images of prenatal studies were selected from 13 cases to illustrate different scenarios and the recommendations given. In addition, a review of the literature was performed to support our advice to parents.
Eleven patients were female and two patients were male. The postnatal diagnoses were cloacal exstrophy (6), cloaca (5), posterior cloaca variant (1), and covered cloacal exstrophy (1). The selected abnormal prenatal imaging findings in these 13 patients included hydronephrosis (12), neural tube defect (8), omphalocele (7), lack of meconium at expected rectal location (7), vertebral anomaly (7), non-visualize bladder (5), distended bladder (5), hydrocolpos (4), dilated or echogenic bowel (3), umbilical cord cyst (3), separated pubic bones (2), and the “elephant trunk” sign (2). The prenatal diagnosis was correct in 10 cases, partially correct in two cases, and it was missed in one case. All parents received prenatal counseling depending on the specific diagnosis.
The continuous technologic innovations in prenatal imaging make it possible to prenatally diagnose more complex anomalies including cloaca and cloacal exstrophy with increased levels of confidence and enhance the benefit of prenatal counseling. Together, these allow the parents to be better prepared for the condition and the care team to provide the best possible initial management in order to improve the outcomes of these challenging patients.
Prenatal diagnosis; Anorectal malformation; Cloaca; Cloacal exstrophy; Covered exstrophy; Posterior cloaca
To evaluate our results of definitive repair of anorectal malformations in patients with delayed presentation, during and beyond adolescence.
Material and Methods:
It is a retrospective analysis of all adolescent patients presenting for the first time for definitive repairs and innate patients - colostomy performed during the neonatal period, but who had lost to follow-up. It includes 15 patients (2 male and 13 female) aged from 13 to 32 years. Three well-decompressed female patients were managed by primary anterior sagittal anorectoplasty (ASARP). Twelve patients underwent staged procedures. Five patients (two male and three female) underwent posterior sagittal anorectoplasty (PSARP). The oldest male patient underwent abdominal-PSARP.
All of them attained socially acceptable fecal continence at follow-up of 1–4½ years. They are satisfied with the functional and cosmetic outcome of repair of their anomalies.
Prospects of fecal continence are good when definitive repair of anorectal malformations is done by an expert, even in the adolescent age group and beyond.
Anorectal malformations; anovestibular fistula; delayed presentation; pouch colon
The rate of continent urinary diversion after radical cystectomy for bladder cancer varies by patient and provider characteristics. Demonstration of equivalent complication rates, independent of diversion type, may decrease provider reluctance to perform continent reconstructions. The authors sought to determine whether continent reconstructions confer increased complication rates after radical cystectomy.
From the Nationwide Inpatient Sample, the authors used International Classification of Disease (ICD-9) codes to identify subjects who underwent radical cystectomy for bladder cancer during 2001–2005. They determined acute postoperative medical and surgical complications from ICD-9 codes and compared complication rates by reconstruction type using the nearest neighbor propensity score matching method and multivariate logistic regression models.
Adjusting for case-mix differences between reconstructive groups, continent diversions conferred a lower risk of medical, surgical, and disposition-related complications that was statistically significant for bowel (3.1% lower risk; 95% confidence interval [95% CI], −6.8% to −0.1%), urinary (1.2% lower risk; 95% CI, −2.3%, to −0.4%), and other surgical complications (3.0% lower risk; 95% CI, −6.2% to −0.4%), and discharge other than home (8.2% lower risk; 95% CI, −12.1% to −4.6%) compared with ileal conduit subjects. Older age and certain comorbid conditions, including congestive heart failure and preoperative weight loss, were associated with significantly increased odds of postoperative medical and surgical complications in all subjects.
Mode of urinary diversion after radical cystectomy for bladder cancer is not associated with increased risk of immediate postoperative complications. These results may encourage broader consideration of continent urinary diversion without concern for increased complication rates.
bladder cancer; radical cystectomy; morbidity; urinary diversion
Two patients are presented in whom ischaemic colitis followed some years after abdomino-perineal excision of the rectum for carcinoma. The first patient was a young man without evidence of arterial disease and the second patient suffered from auricular fibrillation, thought to be due to ischaemic heart disease.
Ligation of the inferior mesenteric artery in the operation of abdomino-perineal excision of the rectum may reduce the blood flow through the marginal artery of Drummond rendering the remaining colon more liable to ischaemic damage.
Patients who pass bright blood through a colostomy following abdomino-perineal excision of the rectum for carcinoma may have ischaemic colitis rather than a recurrence of the neoplasm.
A study on the response of the external anal sphincter (EAS) to the passage of urine through the urethra during micturition could not be found in the literature. We investigated the hypothesis that urine passage through the urethra effects EAS contraction to guard against possible flatus or stool leakage during micturition.
The study was performed in 23 healthy volunteers (age, 38.6 ± 10.8 [SD] years; 14 men and 9 women). The EAS electromyogram (EMG) was performed during micturition by surface electrodes applied to the EAS. Also, the EAS EMG response to urethral stimulation by a catheter-mounted electrode was registered. The test was repeated after individual anesthetization of the EAS and urethra.
The EAS EMG recorded a significant increase (P < 0.01) during micturition and on urethral stimulation at the bladder neck. Stimulation of the prostatic, membranous, or penile urethra produced no significant change in the EAS EMG. Urethral stimulation after individual EAS and urethral anesthetization did not cause any changes in the EAS EMG.
Urine passing through the urethra or urethral stimulation at the vesical neck produced an increase in the EAS EMG, which presumably denotes EAS contraction, which seems to guard against flatus or fecal leakage during micturition. EAS contraction on urethral stimulation is suggested to be mediated through a urethro–anal reflex. Further studies on this issue may potentially prove the diagnostic significance of this reflex in micturition and defecation disorders.
Electromyography; Sphincter reflex; Flatus; Stools; Urethra; Defecation; Micturition
BACKGROUND Cloacal exstrophy is a rare, complex defect of the entire pelvis and its contents that occurs during embryogenesis and is associated with severe phallic inadequacy or phallic absence in genetic males. For about 25 years, neonatal assignment to female sex has been advocated for affected males to overcome the issue of phallic inadequacy, but data on outcome remain sparse.
METHODS We assessed all 16 genetic males in our cloacal-exstrophy clinic at the ages of 5 to 16 years. Fourteen underwent neonatal assignment to female sex socially, legally, and surgically; the parents of the remaining two refused to do so. Detailed questionnaires extensively evaluated the development of sexual role and identity, as defined by the subjects' persistent declarations of their sex.
RESULTS Eight of the 14 subjects assigned to female sex declared themselves male during the course of this study, whereas the 2 raised as males remained male. Subjects could be grouped according to their stated sexual identity. Five subjects were living as females; three were living with unclear sexual identity, although two of the three had declared themselves male; and eight were living as males, six of whom had reassigned themselves to male sex. All 16 subjects had moderate-to-marked interests and attitudes that were considered typical of males. Follow-up ranged from 34 to 98 months.
CONCLUSIONS Routine neonatal assignment of genetic males to female sex because of severe phallic inadequacy can result in unpredictable sexual identification. Clinical interventions in such children should be reexamined in the light of these findings.
Symptomatic perineal hernias following abdomino-perineal excision of rectum have been reported to occur uncommonly. We present the case of a 79-year-old gentleman who developed a perineal hernia after laparoscopic-assisted extralevator abdomino-perineal excision (ELAPE) of the rectum. Despite initial myocutaneous flap repair, there was further symptomatic recurrence. Magnetic resonance imaging demonstrated non-compromised bowel extending beneath the gracilis flap with extension into the adductor compartment of the left thigh. Given the recurrent nature, a rectus flap repair was performed and after 15 months, he remains hernia free. There is currently no consensus as to the optimal operative technique in the prevention and management of these hernias; however, primary reconstruction at the time of ELAPE may be preferable. Symptomatic perineal hernias can be severely debilitating and require operative repair. We suggest that surgical options should be discussed and carried out with the input of a Plastic surgeon.
Fourteen patients with progressive autonomic failure and multiple system atrophy have been investigated by urodynamic, electromyographic and neurohistochemical means and the results compared with a series of age-matched controls. Three fundamental abnormalities of lower urinary tract function have been identified: (1) Involuntary detrusor contractions in response to bladder filling. It is suggested that these may be the result of a loss of inhibitory influences from the corpus striatum and substantia nigra. (2) Loss of the ability to initiate a voluntary micturition reflex. This may reflect the degeneration of neurons in pontine and medullary nuclei and in the sacral intermediolateral columns. In addition, these studies have demonstrated a significant reduction in the density of acetylcholinesterase-containing nerves in bladder muscle. (3) Profound urethral dysfunction. This appears to be partly due to a loss of proximal urethral sphincter tone, which causes bladder neck incompetence. In addition, the function of the striated component of the urethral sphincter is impaired. Individual motor units recorded from this muscle were clearly abnormal when compared with controls and suggested that reinnervation had occurred. We suggest that this is the result of degeneration of a specific group of sacral anterior horn cells known as Onuf's nucleus. The evidence that these particular motor units are affected, while others are spared, poses fundamental questions about the nature of selective vulnerability in degenerative diseases of the nervous system.
This study analyses the morbidity and mortality of colostomy formation and closure over a 17-year period during which 138 consecutive infants and children had a colostomy formed as the initial management of Hirschsprung's disease or anorectal malformation. Complications after colostomy formation were encountered in 38 (27.5%) patients and included colostomy prolapse, stenosis, retraction, dysfunction, skin excoriation and parastomal hernia. The complication rate with transverse colostomies was higher than with other types. Colostomy closure was associated with complications in nine patients (6.5%), the most serious of which was adhesive small bowel obstruction (5). The mortality was less than 1%, but significant morbidity still exists. Refinements in surgical technique may help reduce the incidence of complications, but stoma prolapse, particularly with transverse colostomies, remains a major challenge.
This study reviewed the impact of pre-operative chemoradiotherapy or post-operative chemotherapy and/or radiotherapy on total mesorectal excision (TME) for ultralow rectal cancers that required either low anterior resection with peranal coloanal anastomosis or abdomino-perineal resection (APR). We examined surgical complications, local recurrence and survival.
Of the 1270 patients who underwent radical resection for rectal cancer from 1994 till 2007, 180 with tumors within 4 cm with either peranal coloanal anastomosis or APR were analyzed. Patients were compared in groups that had surgery only (Group A), pre-operative chemoradiotherapy (Group B), and post-operative therapy (Group C).
There were 115 males and the mean age was 65.43 years (range 30-89). APR was performed in 134 patients while 46 had a sphincter-preserving resection with peranal coloanal anastomosis. The mean follow-up period was 52.98 months (range: 0.57 to 178.9). There were 69, 58 and 53 patients in Groups A, B, and C, respectively. Nine patients in Group B could go on to have sphincter-saving rectal resection. The overall peri-operative complication rate was 43.4% in Group A vs. 29.3% in Group B vs. 39.6% in Group C, respectively. The local recurrence rate was significantly lower in Group B (8.6.9% vs. 21.7% in Group A vs. 33.9% in Group C) p < 0.05. The 5-year cancer-specific survival rates for Group A was 49.3%, Group B was 69.9% and Group C was 38.8% (p = 0.14).
Pre-operative chemoradiation in low rectal cancer is not associated with a higher incidence of peri-operative complications and its benefits may include reduction local recurrence.
The past several decades have seen multiple advances in the surgical reconstruction for girls born with Disorders of Sexual Differentiation. This surgery can be technically very demanding, and must be individualized for each patient, as the degree of virilization and level of confluence of the vagina and urogenital sinus will dictate the surgical approach.
In this manuscript we present our approach and experience in the surgical options for girls born with Congenital Adrenal Hyperplasia, with special attention regarding clitoroplasty, urogenital mobilization, and vaginoplasty.
Congenital adrenal hyperplasia; feminizing genitoplasty; urogenital sinus; vaginoplasty
Male to female transsexuals frequently seek feminizing vaginoplasty for “below the waist” conformation, enhancement of sexual identity, and interactive sexual function. The author shares his experience with his first 250 primary surgical procedures. Included is a brief historical background, the patient selection process, some guidelines from the World Professional Association for Transgender Health (standards of care), preoperative evaluation and instructions, surgical technique, postoperative regimen, risk factors, results, complications and management. The patients all underwent feminizing vaginoplasty at the author's ambulatory surgical facility, which includes an overnight stay. The author's results suggest that feminizing vaginoplasty when performed vigilantly on a select group of patients is feasible.
Feminizing vaginoplasty; male to female transsexual surgery; labiaplasty; perineal urethrostomy; total penectomy; clitoroplasty; orchiectomy
Arteriovenous fistula is defined as an abnormal communication between the arterial and venous systems. The complexity of congenital arteriovenous malformations makes treatment challenging. We present the case of a 23-year-old woman who had a complex congenital arteriovenous malformation in her left leg and a history of 2 unsuccessful coil-embolization procedures. We ligated all the feeding arteries of the arteriovenous malformation in the region of the superficial femoral artery, and the surgery was successful without sequelae. The patient returned 2 years later with thrombosis of the great saphenous vein and underwent a second operation. The thrombosed vein and all varicosities were excised successfully.
Surgery can be an effective method for correcting complex congenital arteriovenous malformations, especially in the lower limbs. A 2-staged surgical approach like ours might be a good option in suitable patients.
Arteriovenous fistula/surgery; arteriovenous malformations/diagnosis/surgery; blood vessels/abnormalities; femoral artery/surgery; leg/blood supply; treatment outcome
Malformations of the middle ear are classified as minor and major malformations. Minor malformations appear with regular external auditory canal, tympanic membrane and aerated middle ear space. The conducting hearing loss is due to fixation or interruption of the ossicular chain. The treatment is surgical, following the rules of ossiculoplasty and stapes surgery. In major malformations (congenital aural atresia) there is no external auditory canal and a deformed or missing pinna. The mastoid and the middle ear space may be underdevelopped, the ossicular chain is dysplastic. Surgical therapy is possible in patients with good aeration of the temporal bone, existing windows, a near normal positioned facial nerve and a mobile ossicular chain. Plastic and reconstructive surgery of the pinna should proceed the reconstruction of the external auditory canal and middle ear. In cases of good prognosis unilateral aural atresia can be approached already in childhood. In patients with high risk of surgical failure, bone anchored hearing aids are the treatment of choice. Recent reports of implantable hearing devices may be discussed as an alternative treatment for selected patients.
middle ear malformation; congenital oral atresia; surgical treatment; bone anchored hearing aid; implantable hearing aid
Background: Treatment of high anal fistulas may be associated with a high risk of continence disorders. Beside traditional procedure of flap-reconstruction the occlusion of the fistula tract using fistula-plugs offers a new sphincter-saving treatment option. In this study for the first time results from Germany are described.
Patients and method: 40 patients (30 male, 10 female, age 51±12 years) underwent closure of a high trans-sphincteric (n=28) or supra-sphincteric (n=12) fistula with Gore BioA Fistula Plug® in three surgical departments. The surgical procedures had been performed by five colorectal surgeons. Four patients had Crohn’s disease. Preoperatively 33 patients were completely continent; seven patients complained of minor continence disorders. Treatment of the patients was performed on a intent-to-treat basis and evaluation of the results was retrospective using pooled data from each center.
Results: Postoperatively one patient developed an abscess, which had to be managed surgically. In two patients the plug had fallen out within the first two weeks postoperatively. Six months after surgery the fistula has been healed in 20 patients (50.0%). Three additional fistulas healed after 7, 9 rsp. 12 months. The overall healing-rate was 57.5% (23/40). The healing rate differs considerably between the surgeons from 0 to 75% and depends on the number of previous interventions. In patients having only drainage of the abscess success occurred in 63.6% (14/22) whereas in patients after one or more flap fistula reconstruction the healing rate decreased slightly to 50% (9/18). No patient complained about any impairment of his preoperative continence status.
Conclusion: By occlusion of high anal fistulas with a plug technique definitive healing could be achieved in nearly every second patients. Previous surgery seems to have a negative impact on success rate. We have not observed any negative impact on anal continence. From that point of view anal fistula plugs might be discussed as a treatment option for high anal fistulas, but further studies are needed to gain conclusive evidence.
complex fistula-in-ano; transsphincteric fistula; suprasphincteric fistula; surgical procedures; fecal incontinence; fistula plug