Myofascial wrap to treat intractable urinary incontinence in children

OBJECTIVES: The management of intractable urinary incontinence in the patient with cloacal or bladder exstrophy/epispadias, failed bladder neck plasty, or failed augmentation cystoplasty remains a surgical challenge. The myofascial wrap, a modification of the rectus fascial wrap, was developed to treat intractable urinary incontinence due to sphincteric incompetence in these problematic cases. A full-thickness, vascularized pedicle of anterior rectus sheath, rectus abdominis muscle, and posterior rector sheath is incorporated into a bladder neck wrap to provide support, mucosal coaptation, and active muscular tone. METHODS: Eight patients (5 females and 3 males) with total urinary incontinence due to sphincteric incompetence underwent the myofascial wrap. Urinary tract pathology included cloacal exstrophy (2), female epispadias (2), classic bladder exstrophy (1), male epispadias (1), myelomeningocele (1), and a pelvic tumor (1). The procedure is performed by harvesting a full-thickness strip of pedicled rectus muscle along with the anterior and posterior fascial sheaths. The strip is passed underneath and then over the bladder neck in a near 360 degrees wrap. The free end of the wrap is anchored into the pubic bone in an ipsilateral subperiosteal pouch. RESULTS: Six of the 8 patients are completely continent, and 2 patients void spontaneously without the need for catheterization. CONCLUSIONS: The myofascial wrap provides support, mucosal coaptation, and muscular tone to an incompetent sphincter and bladder neck. Favorable results in a very difficult population of pediatric patients warrant its continued use.     

Massive ovarian enlargement in primary hypothyroidism

OBJECTIVE: To report a case of ovarian cyst formation and myxedematous infiltration of the ovary in a subject with primary hypothyroidism. DESIGN: Retrospective case report. SETTING: University hospital. PATIENT(S): A 16-year-old female adolescent with pelvic pain, galactorrhea, irregular menses, and ovarian cysts on pelvic examination. INTERVENTION(S): Laparotomy with bilateral ovarian wedge resection and thyroid replacement therapy. MAIN OUTCOME MEASURE(S): Ovarian histopathology, thyroid function tests, and menstrual history. RESULT(S): Resolution of patient's pain, galactorrhea, and resumption of normal menses. CONCLUSION(S): Ovarian cyst formation may accompany primary hypothyroidism in the child with accelerated or delayed sexual maturation. To date, the underlying pathophysiology of the morphological changes in the ovary remain enigmatic. This case report provides the first insight into the actual histologic changes that occur in ovaries of subjects with primary hypothyroidism without secondary ovarian pathology such as torsion. There is clear evidence of myxedematous infiltration into the ovarian stroma without luteinization of the theca interna. These microscopic findings suggest that local changes occurring independent of gonadotropin stimulation may contribute significantly to altered morphology of the ovaries in primary hypothyroidism.     

Current urologic management of cloacal exstrophy: experience with 11 patients

PURPOSE: Since 1980 the authors have treated 12 infants with cloacal exstrophy (10 classical and 2 variants). Eleven patients had repair, and are all surviving. The initial phases of management that led to improved survival have previously been reported. Quality of life is now a major focus for the cloacal exstrophy patient. During the past 10 years, nine of the 11 patients had lower urinary tract reconstructive procedures. This review evaluates experience with reconstructive efforts to achieve bowel and bladder control and to improve the quality of life in this complex group of patients. METHODS: Through review of patient charts and by patient interviews, data were collected to evaluate the ability to provide urinary and bowel control. A continence score was applied to provide a measure of success: voluntary control, 3; control with an enema program or intermittent catheterization, 2; incontinence with a well-functioning stoma, 1; and incontinence without a stoma, 0. The best continence score is 6 (genitourinary and gastrointestinal). Surgical complications, urodynamic and metabolic sequelae of continent urinary diversion were reviewed. RESULTS: At the time of the authors' previous report, eight of 11 patients had a continence score of 2 or less. Currently, eight of 11 patients have a score of 3 or better (five with enteric stoma and continent urinary diversion, two with enema program and continent urinary diversion, and one with enema program and continent bladder). Urinary-diversion procedures have included two gastric augmentations and five gastric reservoirs, two of which have required subsequent bowel augmentation. Gastric augmentations carry a definite risk of metabolic problems with three of our patients demonstrating significant episodes of metabolic alkalosis. In addition, results of urodynamic monitoring suggests that gastric reservoirs may be less compliant than reservoirs formed using other bowel segments. CONCLUSIONS: Modern principles of continent urinary diversion have been successfully applied to the cloacal exstrophy patient further improving their quality of life. Use of gastric flaps with preservation of intestinal length has been central to urologic reconstructive efforts. Use of stomach alone for formation of urinary reservoirs may produce suboptimal compliance, and composite ileogastric construction should be considered if the gastric flap is of marginal size.     

Cloaca, the most severe degree of imperforate anus: experience with 195 cases

OBJECTIVE: To provide a follow-up of 195 patients with cloacal malformations seen by the author from 1959 to 1998. SUMMARY BACKGROUND DATA: Cloaca, which occurs in approximately 1 of 50,000 births, is the most complex type of imperforate anus with confluence of the rectum, vagina, and bladder in a urogenital sinus. Functional results for the bowel, the genital tract, and the urinary tract were formerly poor. Cloacal exstrophy, which is an even more complex spectrum of malformations, was uniformly fatal until 1960. In addition to imperforate anus, these babies have an omphalocele, two exstrophic bladders, between which there is an open cecum, and a blindly ending colon hanging down in the pelvis from the cecum. Although both of these diagnoses contain the word "cloaca," which is Latin for sewer, they are really two separate entities in terms of surgical management. Cloaca and cloacal exstrophy in most cases are very different anatomic problems. However, there are variants that are like a hybrid, which is the rationale for reporting together an experience with both entities. METHODS: Records were reviewed of 154 patients with cloaca and 41 patients with cloacal exstrophy to assess anorectal function, urinary continence, and sexual function where available. RESULTS: Follow-up was available in 141 cloaca patients: 82 have spontaneous bowel movements and satisfactory control, 38 use enemas to evacuate, 9 have a colostomy, 7 have fecal soiling, and 5 are too recently operated to evaluate. Regarding urinary control, 83 void spontaneously, 40 catheterize to empty, 4 have urinary diversion, 1 has a continent diversion, 5 patients are wet, and 8 are too recently operated to judge. Twenty-four patients are now adults, 17 of who have experienced coitus and 7 have not. Seven have had babies, all except one by cesarean section. Results of surgery for cloacal exstrophy are not as good, but are encouraging nonetheless for an anomaly that was uniformly fatal before 1960. Of the 41 cloacal exstrophy patients being followed, 7 have not undergone surgery. Fifteen have a colostomy; 19 had pull-through of the colon, but 3 were subsequently reversed for fecal incontinence. Most depend on enemas to evacuate. Urinary dryness was attained in 30 patients, usually by intermittent catheterization of the bladder, which was augmented with small bowel or stomach or both. Only three void voluntarily. Fifteen of the completed long-term patients wear no bag. Only three of the completed patients wear two bags. The rest have one bag. CONCLUSIONS: Imperforate anus and associated malformations in cloaca and cloacal exstrophy are not hopeless problems. A reasonable lifestyle can be achieved for most of these children with comprehensive surgical planning.     

Dysraphic myelodysplasias associated with urogenital and anorectal anomalies: prevalence and types seen with MR imaging

OBJECTIVE: The purpose of this study was to determine by MR imaging the prevalence and types of dysraphic abnormalities of the spinal cord (i.e., myelodysplasias) associated with urogenital and anorectal malformations of childhood. MATERIALS AND METHODS: Since 1987, 92 patients with imperforate anus complex, cloacal malformation, and cloacal exstrophy have had MR imaging as a screening examination for occult dysraphic myelodysplasia. The prevalence and types of myelodysplasia were determined for each group. RESULTS: The prevalence of dysraphic myelodysplasia in each group of children was 17% (1/6) for low imperforate anus (ectopic anus), 34% (11/32) for high imperforate anus (with fistulization), 46% (19/41) for cloacal malformation, and 100% (13/13) for cloacal exstrophy. The most common abnormalities in each group were tethered cord with intradural or filar lipoma in imperforate anus; low-placed or dysplastic conus medullaris and tethered cord with lipoma or myelolipoma in cloacal malformation; and lipomyelocele, lipomyelomeningocele, or lipomyelocystocele in cloacal exstrophy. CONCLUSION: Our results show that the prevalence of myelodysplasia as seen on MR imaging is high in patients with urogenital and anorectal anomalies.     

Toward easier endoscopy--the Oriental perspectives

This development of pathology in conserved ovaries is defined as the residual ovary syndrome (ROS). It consists of pelvic pain, pelvic mass, dyspareunia as a single or a cluster of symptoms. It is estimated that at least 5% of patients will develop ROS and require surgery for it following hysterectomy. We describe a case of ROS in a 41-year-old woman who developed an abdominopelvic mass the size of a pregnancy of 24 weeks' gestation within 11 weeks of hysterectomy and right salpingo-oophorectomy. At laparotomy, the mass was found to be a huge benign ovarian cyst measuring 11.5 x 11.0 x 14.1 cm. Histology showed benign thick-walled follicular cysts. ROS can occur 'acutely', our case having the shortest latent period of onset ever reported.     

Scanning electron microscopy studies of the human lens capsule after capsulorhexis

Choledochal cysts are malformations of the biliary ductal system, which rarely occur in infancy. In neonates and infants, choledochal cysts are congenital, may be associated with distal biliary atresia and typically presents with cholestatic jaundice and acholic stools characteristic of biliary obstruction. In older children and adults, it may be acquired in association with an anomalous pancreaticobiliary union and presents with any combination of intermittent jaundice, abdominal pain, and a palpable abdominal mass. Early detection of choledochal cysts is important in the prevention of the fatal complications of biliary obstruction. Here, we present two cases of congenital choledochal cyst in which the patients were 9 weeks old and 7 weeks old respectively. Both of them presented with jaundice, acholic stools, and hepatomegaly. Combined use of sonography, computed tomography and hepatobiliary scintigraphy demonstrated the presence of choledochal cysts. Thereafter, they underwent surgical excision of the cysts with Roux-en-Y reconstruction of the biliary tree; the results were successful.     

Bilateral ureteral obstruction and renal failure caused by massive retroperitoneal hematoma: is there a pelvic compartment syndrome analogous to abdominal compartment syndrome?

OBJECTIVES: To describe an intrapelvic compartment syndrome analogous to abdominal compartment syndrome and to characterize its diagnosis and treatment. DESIGN: Retrospective analysis. SETTING: Level I trauma center. PATIENTS: Three patients with pelvic ring or acetabular fractures presented with bilateral ureteral obstruction, renal organ failure, and anuria due to direct compression of both ureters in the true pelvis by a massive retroperitoneal hematoma. INTERVENTION: Surgical therapy consisted of fracture stabilization, decompression of the retroperitoneal space, and evacuation of the hematoma. Persistent isolated bleeding points were either embolized preoperatively or ligated. RESULTS: After decompression, all three patients promptly recovered their renal organ function. CONCLUSION: An intrapelvic compartment syndrome can be defined as bilateral ureteral obstruction and renal failure caused by a massive intrapelvic hematoma with increased retroperitoneal pressure. Diagnostic differentiation of anuria in patients with pelvic ring or acetabular fractures must include intrapelvic compartment syndrome. Early diagnosis and treatment are mandatory.     

Characterization of mutations at the mouse phenylalanine hydroxylase locus

By using a three-dimensional computed tomography (CT) scanner, we compared the anatomic features of the pelvis of three fetuses of same gestational age, one with a normal pelvis representing the reference model, one with classic bladder exstrophy, and one with cloacal exstrophy. The tomography slices were selected at the same levels for each case. Three angles expressing external opening of the pelvis were defined. Comparing normal and abnormal pelvises allowed definition of three criteria for the correction of the malformation: (a) the sum of the differential angles gives the amplitude of the correction needed; (b) a supraacetabular osteotomy appears to allow best closure of the pelvic ring; (c) only three slices of a CT scan are needed, which cannot be harmful, especially for neonates. Therefore, we believe that a CT scan of the pelvis should be performed whenever an osteotomy is planned in the surgical reconstruction of bladder and cloacal exstrophy.     

Induction of early and bioeffective antibody response in rodents with the luteinizing hormone-releasing hormone vaccine given as a single dose in biodegradable microspheres along with alum

Total urinary incontinence is a difficult problem faced by the urologist. Several techniques to increase ureteral resistance have been described. The majority of them rely on intermittent catheterization for bladder emptying, especially in neurogenic incontinence. We have developed a new procedure in which a bladder flap is used to create a neourethra. This urethral extension acts as a flap valve to provide continence. Bladder emptying is accomplished by clean intermittent catheterization. Urethral lengthening with an anterior bladder-wall flap was performed in 18 patients aged a mean of 8.9 years who had neurogenic incontinence (14) or exstrophy (4). Patients with previous bladder interventions received a lateralized anterior flap. Bladder augmentation was performed in 14 of the 18 patients [detubularized ileum (11), detubularized colon (3)]. The average follow-up period is currently 29.3 months. Continence was achieved in 13 of the 18 patients (72%). Complications included urethrovesical fistulae, which developed in two patients. Two patients could not perform catheterization due to pain but had no obstruction to passage of catheter (exstrophy). Ureteral lengthening with an anterior bladder-wall flap is a useful alternative for the surgical treatment of urinary incontinence. This technique achieves a good continence rate and presents few problems with catheterization.     

Melanosis peritonei associated with enteric duplication cyst. A case report

Melanosis peritonei is extremely rare, and only five cases have been reported in the English literature, four in association with ovarian dermoid cysts, and one with a peritoneal cyst. We describe an additional case occurring in a girl two years of age who also had an enteric cyst. This is the first reported case of melanosis peritonei not associated with an ovarian teratoma, but with an enteric duplication cyst. Melanophages were present focally in the submucosa, superficial muscle layer, and ulcer bases of the cyst and extensively as small nodules on the peritoneal surface and in the omentum. The tendency of perivascular nodular aggregation of melanophages favors a hematogenous (or lymphatic) spread rather than implantation. Although clear evidence of melanocytic aggregation is lacking, are speculate that the melanin originated from the esophageal-like squamous mucosa of the cyst.     

Diagnosing the combination of renal dysgenesis, Gartner's duct cyst and ipsilateral müllerian duct obstruction

PURPOSE: We describe the differential points in the diagnosis of the combination of renal dysgenesis, Gartner's duct cyst and ipsilateral müllerian duct obstruction. Various imaging studies and urological procedures were performed. We report our experience in detecting these anomalies in 10 girls and review the literature. MATERIALS AND METHODS: Ten girls, 7 to 13 years old, with this combination of anomalies were identified in the last 10 years. Imaging studies as well as urological procedures were selectively performed, especially at puberty following menarche. Patients received long-term followup with ultrasound. RESULTS: Cystic dilation of Gartner's duct protruded into the bladder and presented as a ureterocele in 5 patients and posterior to the bladder in 5. Surgical removal of a partial portion of a Gartner's duct cyst was performed in 5 patients for alleviation of urinary symptoms. Unilateral müllerian duct obstruction was demonstrated in all 10 patients. Excision of the vaginal septum was performed in 6 patients for relief of genital obstruction. CONCLUSIONS: When cystic dilatation of the pelvis, especially a ureterocele-like cyst without ureteral dilatation, is found in girls with ipsilateral renal dysgenesis, the possibility of a Gartner's duct cyst should be considered. For early detection and treatment of unilateral obstruction of duplicated müllerian ducts pelvic sonography should be performed at puberty, especially just after menarche, in girls with renal dysgenesis and ipsilateral Gartner's duct cyst.     

Sonographic features of ovarian remnants

Ovarian remnants occur after a portion of ovarian tissue is left behind unintentionally after oophorectomy. The ovarian remnant may be functional and cystic, producing pelvic pain and, in some patients, extrinsic compression of the distal ureter. Ovarian remnants frequently are associated with adhesions from previous pelvic surgery for endometriosis or pelvic inflammatory disease. Ovarian remnants also may be included within pelvic peritoneal inclusion cysts. In this retrospective study, the sonographic features of ovarian remnants in 10 patients with surgical proof or clinical follow-up data are described. Most ovarian remnants were simple cysts (seven of 10), three had multiple septations, and six had a rim of presumably ovarian tissue with arterial and venous flow. Three patients with ovarian remnant masses that were aspirated had symptomatic relief without recurrence. In one patient, guided aspiration was unsuccessful, probably owing to the presence of organized hemorrhage within the mass. Extrinsic compression of the distal ureter was observed in one patient, who was treated with gonadotropin releasing hormone agonist (Lupron). The sonographic findings of a completely cystic or multiseptated pelvic mass with a rim of vascularized solid tissue in a postoophorectomy patient, although such cases are rare, suggest the diagnosis of an ovarian remnant. If the diagnosis can be established with a high degree of certainty, sonographically guided aspiration may be attempted in an effort to provide symptomatic relief. Otherwise, sonography is useful in serial assessment of these masses in patients receiving medical treatment.     

Hydatid cyst of Morgagni: any impact on fertility?

On laparoscopy in 3 patients with primary infertility, the only pathology found was hydatid cysts of Morgagni that were excised. In one patient with monolateral hydatid cyst, pregnancy failed to be achieved. Despite prior failure of repeated trials of ovulation induction and intrauterine insemination in the other 2 patients, a spontaneous pregnancy was achieved within 2-3 months following laparoscopic extirpation of hydatid cysts of Morgagni. The hydatid cysts were bilateral in one case; and monolateral (in relation to the only present tube with a unicornuate uterus) in the other case. It is concluded that hydatids of Morgagni, as a single pelvic pathology, might hinder fertility. Laparoscopic extirpation of these cysts would improve ovum pick-up and enhance fertility.     

Thalidomide increases both REM and stage 3-4 sleep in human adults: a preliminary study

OBJECTIVE: Methotrexate has been documented to accumulate in pleural effusions and ascitic fluid, resulting in severe local and systemic toxicity. In the following case report, we publish results of intraoperative measurements of methotrexate levels in serum and an ovarian cyst and attempt to determine if ovarian cysts similarly act as a depot for methotrexate. METHODS: After determining intraoperative measurements of serum and ovarian cystic levels of methotrexate, we compared demonstrated pharmacokinetics to those expected by using pharmacokinetic systems analysis software. RESULTS: Intraoperative measurement of methotrexate levels on day 3 of a 5-day methotrexate regimen revealed a serum methotrexate concentration of 1.6 x 10(-7) M and a concentration of 3.1 x 10(-7) M within the 166.4 ml ovarian cyst. CONCLUSIONS: The measured levels demonstrate that methotrexate is sequestered within an ovarian cyst resulting in higher local drug levels. Our pharmacokinetic analysis suggests that methotrexate doses less than 100 mg/m2 can be safely administered to patients with small ovarian cysts. However, computed simulations support the possibility of local and systemic toxicity arising from large ovarian cysts when using high doses of methotrexate.     

Primary infertility in a phenotypic male with 46XX chromosomal constitution

The case of a 32 year old male with normal male adrenarchal hair pattern, bilateral gynaecomastia, a small phallus, hypospadias and bilateral poorly developed testes presenting with primary infertility secondary to azoospermia and a pelvic cyst is described. Repeated chromosomal analysis showed 46XX chromosomal constitution. Laparotomy revealed a simple cyst between the urinary bladder and the rectum. XX male syndrome is a rare cause of male infertility. The majority of cases is due to interchange of a fragment of the short arm of the Y chromosome containing the region that encodes the testes determining factor with the X chromosome. The presence of a simple cyst in the anatomical location of the uterus to our knowledge has not been reported in the literature.     

Cervical arachnoid cysts after craniocervical decompression for Chiari II malformations: report of three cases

OBJECTIVE AND IMPORTANCE: We describe three cases in which ventrally situated cervical arachnoid cysts led to spinal cord or cervicomedullary compression after repeat craniocervical decompression for Chiari II malformations. CLINICAL PRESENTATION: All three patients underwent craniocervical decompression when their Chiari malformations became symptomatic. The first patient developed chronic vertiginous spells and headache and was treated with repeated craniocervical decompression procedures during several years. Seven months after undergoing her third decompression procedure, she developed severe dizzy spells, which were determined to be of brain stem origin. The second patient had a small, asymptomatic arachnoid cyst anterior to the brain stem discovered at age 6 years. After undergoing repeat craniocervical decompression for headaches 8 years after undergoing his first procedure, the patient developed severe neck pain and acute quadraparesis. A third patient underwent repeat craniocervical decompression at age 14 years for cranial nerve dysfunction. Postoperatively, he acutely developed paresis of extraocular movements and incoordination of the upper extremities. All three patients were found to have anteriorly situated arachnoid cysts compressing the brain stem and/or cervical spinal cord. INTERVENTION AND TECHNIQUE: Fenestration of the arachnoid cyst or drainage with cystoperitoneal shunting adequately treated acute brain stem or cervical spinal cord compression. All three patients had achieved satisfactory relief from their acute symptoms of neural compression at their follow-up examinations. CONCLUSION: An association between spinal arachnoid cysts and neural tube defects has previously been reported. However, the development of previously undetected spinal arachnoid cysts after craniocervical decompression was unexpected. We hypothesize that extensive craniocervical decompression may alter the cerebrospinal fluid pressure dynamics in such a way that the anterior subarachnoid space, previously compressed, may dilate. Occasionally, because of perimedullary arachnoiditis, the cerebrospinal fluid may become loculated and act as a mass. Direct fenestration or shunting may successfully treat this problem, and less extensive craniocervical decompression may avoid it.     

Immunoglobulin gene organization and the mechanism of repertoire development

Twenty-two cases of suprascapular nerve entrapment caused by supraglenoid cyst compression were reviewed. Pain and weakness were the presenting symptoms in 14 shoulders and pain alone in 8. Twenty of the cysts were diagnosed by magnetic resonance imaging, and two were confirmed at surgical exploration. Electromyography of 20 shoulders was positive for neurologic involvement for both the infraspinatus and supraspinatus in 4 cases, for the infraspinatus only in 12, and negative in 4. Sixteen shoulders were treated by open excision, arthroscopy, or both. Superior labral lesions were diagnosed in 11 of 12 patients who underwent arthroscopy. At follow-up 10 of the patients who underwent surgery had complete resolution of symptoms, 5 had occasional pain or weakness, and 1 recurrence required a second surgery. Of six patients treated without surgery, two improved and four had no change. Supraglenoid ganglion cysts are common and can easily be diagnosed by magnetic resonance imaging. For patients with symptoms arthroscopy with repair of the superior labral lesion and either arthroscopic debridement or direct open decompression and excision of the cyst is recommended.     

Acute intestinal obstruction in an elderly patient

The correct diagnosis of an acute abdomen in the aged is difficult, because of its varying presentation. Intestinal obstruction as a cause of acute abdomen is five times more common in the elderly as compared to younger patients. Acute intestinal obstruction in elderly patients may be due to intestinal or gynaecologic malignancies, or more frequently to incancerated hernias, peritoneal adhesions or faecal impaction. This case report describes a 90-year old female patient with intestinal obstruction, due to a bilateral torsion of benign ovarian cysts. Urgent surgery was life-saving. This case demonstrates that early decision for adequate therapy can reduce morbidity and mortality, also in very old patients.     

Failure in self care and heart failure, thiamine deficiency in geriatric patients

The ultrasonographic findings of 25 lesions in 23 patients with surgically proven ovarian masses were reviewed. There were 10 cystic teratomas, two simple cysts, two follicular cysts, two mucinous cystadenomas, two NHL, one corpus luteum cyst, one hydrosalpinx, one serous cystadenoma, one yolk sac carcinoma, one dysgerminoma, one embryonal carcinoma, and one mixed form (yolk sac carcinoma, choriocarcinoma). All patients were less than 15 years old. We classified all cases into four patterns: cystic, cystic with mural nodule, mixed, and solid. Eight lesions of the cystic pattern included two simple cysts, two follicular, cysts and one corpus luteum cyst. The other lesions were benign, too. Nine lesions with the cystic with mural nodule pattern consisted of eight cystic teratomas and one mucinous cystadenoma. All lesions were benign. The mixed pattern was seen in four lesions, half of which were malignant, i. e., one embryonal carcinoma and one yolk sac carcinoma. Four lesions with the solid pattern were all malignant masses: one dysgerminoma, two NHL and one mixed form. In this classification, the cystic and cystic with mural nodule patterns are benign, while mixed and solid patterns are highly suggestive of malignancy.