Short-term outcomes of the multi-institutional bladder exstrophy consortium: Successes and complications in the first two years of collaboration.

INTRODUCTION/BACKGROUND: Bladder exstrophy is a rare diagnosis that presents major reconstructive challenges. To increase experience and proficiency in the care of bladder exstrophy (BE), the Multi-Institutional BE Consortium (MIBEC) was formed, with a focus on refining technical aspects of complete primary repair of bladder exstrophy (CPRE) and subsequent care. OBJECTIVE: Outcome measures included successful CPRE (absence of dehiscence), complications, and integrated points of technique and care over the short-term. STUDY DESIGN: Boston Children's Hospital, Children's Hospital of Philadelphia and Children's Hospital of Wisconsin alternately served as the host, with observation, commentary and critique by visiting collaborating surgeons. CPRE with bilateral iliac osteotomy was performed at 1-3 months of age. High-definition video capture of the surgery allowed local and distant broadcast to facilitate real-time observation and teaching, and recording of all procedures. RESULTS: From February 2013 to February 2015, MIBEC participating surgeons performed CPRE on 27 consecutive patients (22 classic BE, five epispadias). There were no dehiscences in 27 patients (0%, 95% CI 0-12.5%). Thirteen girls and 14 boys underwent CPRE at a median age of 2.3 months (range 0.1-51.6). One boy had a hypospadiac urethral meatus at CPRE completion. Hydronephrosis of mild or moderate grade was present postoperatively in eight girls and two boys. Additional results, per gender, are presented in the Summary table below. DISCUSSION: Absence of dehiscence in this cohort was comparable or compared favorably with the literature. However, several girls had significant obstructive complications following CPRE. The rate of bladder outlet obstruction (BOO) in girls was increased compared with published reports. A low complication rate was noted in the boys following CPRE, which was comparable to reports in the literature, and early signs of continence and spontaneous voiding were noted in some boys and girls. Limitations included variation in patient age at presentation, thereby introducing a wide age range at CPRE. Outcome data were limited by short follow-up regarding voiding with continence. CONCLUSION: This collaborative effort proved beneficial regarding significantly increased surgeon exposure to CPRE, refinement of CPRE technique, surgeon learning and expertise. Technical refinement of CPRE is ongoing.

Clinical experience with the double-plate Ahmed glaucoma valve.

PURPOSE: To evaluate the clinical efficacy and safety of the double-plate Ahmed Glaucoma Valve. DESIGN: Multicenter, retrospective observational case series. METHODS: We reviewed 50 eyes in 50 patients treated with the double-plate Ahmed Glaucoma Valve with mean follow-up of 11.7+/-7.9 months. RESULTS: Average intraocular pressure was reduced from 36.0+/-12.4 mm Hg before surgery to 15.8+/-7.7 mm Hg at the most recent follow-up after surgery (P<.001). The number of glaucoma medications was decreased from 3.3+/-0.8 before surgery to 1.4+/-1.4 after surgery (P<.001). The percent of success was 76% of eyes at the latest follow-up. Choroidal effusion occurred in four eyes (8%) and did not require surgical treatment in any eye. CONCLUSIONS: Despite a poor prognosis group of patients in this study, the double-plate Ahmed Glaucoma Valve was effective in lowering intraocular pressure with a low incidence of complications associated with hypotony.

Sacral agenesis and neurogenic bladder: Long-term outcomes of bladder and kidney function.

BACKGROUND: Sacral agenesis (SA) is a rare congenital condition that refers to the absence of part or all of two or more lower sacral vertebral bodies. It can be associated with neurogenic bladder dysfunction that does not necessarily correlate with the level of spinal or skeletal defect. Patients with SA should undergo urodynamic studies (UDS) to guide lower urinary tract (LUT) management. OBJECTIVE: This review aimed to update the present institutional experience since 1981 of this rare patient population with detailed, long-term follow-up of bladder and kidney function. STUDY DESIGN: A single institution, retrospective, IRB-approved review was performed on patients born after January 1, 1981 with an isolated diagnosis of sacral agenesis without spina bifida, and followed with urologic involvement at Boston Children's Hospital. Records were reviewed for demographics, radiologic imaging, UDS including cystometrogram (CMG) and electromyography (EMG), surgery, and blood chemistries. Comparisons were made between groups of patients based on age at diagnosis, with specific focus on renal function and stability of neurogenic bladder lesion. RESULTS: Forty-three patients were identified: 23 female and 20 male. Thirty-seven children (86%) had a known age of diagnosis. Nineteen were diagnosed before 2 months old, including five who were diagnosed prenatally, 11 were diagnosed between 2 and 18 months, and seven were diagnosed after 18 months. All 43 had UDS, with 24 (55.8%) studied at the time of diagnosis (Summary Table). Twenty had serial full UDS, with 30% demonstrating neurourologic instability. None developed end-stage renal disease (ESRD) or required spinal cord detethering. DISCUSSION: Many children with SA appeared to be diagnosed prenatally or early in life; SA was mostly identified during evaluation of associated anomalies. Though UDS aid in urologic management, testing was not routinely utilized at the time of diagnosis. CONCLUSIONS: This review of long-term follow-up in SA patients showed stable LUT and renal function, with minimal risk of progression to ESRD.

Test-retest reliability of the Capute scales for neurodevelopmental screening of a high risk sample: Impact of test-retest interval and degree of neonatal risk.

AIM: Reliable and valid screening and assessment tools are necessary to identify children at risk for neurodevelopmental disabilities who may require additional services. This study evaluated the test-retest reliability of the Capute Scales in a high-risk sample, hypothesizing adequate reliability across 6- and 12-month intervals. METHODS: Capute Scales scores (N = 66) were collected via retrospective chart review from a NICU follow-up clinic within a large urban medical center spanning three age-ranges: 12-18, 19-24, and 25-36 months. On average, participants were classified as very low birth weight and premature. Reliability of the Capute Scales was evaluated with intraclass correlation coefficients across length of test-retest interval, age at testing, and degree of neonatal complications. RESULTS: The Capute Scales demonstrated high reliability, regardless of length of test-retest interval (ranging from 6 to 14 months) or age of participant, for all index scores, including overall Developmental Quotient (DQ), language-based skill index (CLAMS) and nonverbal reasoning index (CAT). Linear regressions revealed that greater neonatal risk was related to poorer test-retest reliability; however, reliability coefficients remained strong. CONCLUSIONS: The Capute Scales afford clinicians a reliable and valid means of screening and assessing for neurodevelopmental delay within high-risk infant populations.

Molecular sieving in suprachoroidal fluid formation in man.

Suproachoroidal fluid (SCF) obtained at the time of the surgical evacuation of a clinically significant choroidal detachment (CD) was analyzed for its chemical and cellular components in four distinct subgroups: (1) CD following cataract surgery, (2) CD (nonhemorrhagic) following glaucoma surgery, (3) CD (hemorrhagic) following glaucoma surgery, and (4) intraoperative CD during glaucoma surgery in patients with elevated episcleral venous pressure. The fluid obtained in groups 1, 2, and 4 was clear and slightly xanthochromic and contained low-molecular-weight substances in concentrations essentially equal to serum. Proteins and other high-molecular-weight substances were present in lesser amounts than in serum. Albumin, alpha1-antitrypsin, and transferrin were present in amounts approximately equal to those in serum, whereas alpha2-macroglobulin, IgM and IgG were decreased. beta-Lipoprotein and beta-complement were absent. It is postulated that this distribution of serum proteins is a manifestation of molecular sieving and is consistent with the existence of an isoporous membrane between the intravascular and suprachoroidal space with a pore diameter of 144 A. In the intraoperative choroidal effusions, there was evidence for exclusion of more of the lower, as well as all of the higher, molecular weight proteins. This suggested that the degree of molecular sieving increased with increasing filtration rate. In the hemorrhagic SCF, the distinctive character of the fluid and the protein concentrations indicated that the integrity of the capillary membrane was mardkedly disrupted, thereby allowing higher-molecular-weight proteins and cellular elements to enter the space.

Suspected multiple primary tumors of the lacrimal and parotid glands.

A 63-year-old man had apparent primary tumors of the lacrimal and parotid glands. Initially an adenocarcinoma of ectopic lacrimal gland origin was suspected following biopsy of a mass in the left orbit. The patient received radiation therapy to this area and showed no recurrence of the original lesion or development of other tumors for a period of 27 months. At that time, the patient returned with a mass in the left preauricular area. This was surgically resected and found to be an adenocarcinoma of the left parotid gland. Both tumors were histologically similar, mucin-secreting adenocarcinomas. There has been no further evidence of tumor growth in an additional three-year period.

Choroidal effusion during glaucoma surgery in patients with prominent episcleral vessels.

Rapid intraoperative choroidal effusion and flattening of the anterior chamber occurred during glaucoma filtering surgery in four young patients with prominent episcleral vessels, elevated episcleral venous pressure, and advanced open-angle glaucoma. Two of these four patients had Sturge-Weber syndrome. Intraoperative release of suprachoroidal fluid (SCF) through a posterior sclerotomy facilitated reformation of the anterior chamber and repositioning of the iris and ciliary body. Posterior sclerostomy performed prior to opening the anterior chamber minimized the above-mentioned untoward series of events. Analysis of SCF and serum demonstrated considerable differences in total protein and individual immunoglobulin levels; this appears to be a manifestation of molecular sieving at the level of the choriocapillaris. Choroidal detachment and postoperative serous retinal detachment are manifestation of this phenomenon.

Probe placement and power levels in contact transscleral neodymium:YAG cyclophotocoagulation.

Transscleral cyclophotocoagulation using a free-running, thermal-mode neodymium:YAG laser with a 2.2-mm-diameter sapphire-tipped contact probe was performed on 27 enucleated human eyes to investigate the effect of probe placement and power levels. We concluded from gross, light, and scanning electron microscopic examinations that a tissue destructive effect on ciliary body and epithelium was produced by positioning the anterior edge of the probe tip 0.5 to 1.0 mm from the visible limbus using a 5- to 9-W power setting for 0.7 second. An 11-W power setting resulted in extensive loss of anatomic integrity of the ciliary body. Limbal probe placement produced significant iris and lens damage while positioning the probe 1.5 mm posterior to the limbus affected primarily pars plana. Damage to the sclera was not observed using this method of cyclophotocoagulation.

The effect of anterior chamber depth on endothelial cell count after filtration surgery.

Eighteen patients undergoing glaucoma filtration surgery underwent specular microscopic examination 1 day prior to surgery and 4 to 6 months after surgery. Patients were evaluated postoperatively for the presence of iridocorneal or lenticular-corneal touch, anterior chamber depth, and inflammation. Ten eyes that maintained their anterior chamber following glaucoma filtration surgery did not have a significant decrease in corneal endothelial cell density. However, eight eyes that developed a shallow anterior chamber with iridocorneal touch had a mean (+/- SD) decrease of 265 +/- 185 cells (12.4%) peripherally and 250 +/- 243 cells (11.6%) centrally in corneal endothelial cell count. None of the patients with iridocorneal touch developed corneal edema after a mean follow-up of 44.4 +/- 18.0 months. Iridocorneal touch after glaucoma filtration surgery is associated with loss of endothelial cells yet appears to be well tolerated by the cornea.

Pupillary block with posterior chamber intraocular lenses.

We present 12 cases of pupillary block associated with extracapsular cataract extraction and posterior chamber intraocular lenses. In one case, pupillary block occurred despite the presence of a surgical iridectomy. The other 11 patients did not have a surgical iridectomy performed. We recommend that a surgical iridectomy or laser iridotomy be performed in all patients who undergo extracapsular cataract extraction with the implantation of posterior chamber intraocular lenses. Ophthalmologists need to be vigilant in watching for the development of pupillary block in these patients, since pupillary block is not a benign event and serious visual loss or disability may occur.

Angiogenesis capacity as a diagnostic marker for human eye tumors.

Solid tumors have the capacity to continuously stimulate the proliferation of new capillaries. Aliquots (0.1 cc) of aqueous humor were aspirated from the anterior chamber of the eye of 38 patients undergoing elective ophthalmologic surgery. The material was lyophilized and then inplanted on the chorioallantoic membrane (CAM) of 10-day-old chick embryos for bioassay. The angiogenesis capacity of each sample was graded as negative or positive. Nine of 10 patients with histologically proven retinoblastoma had positive vascular responses. Seven of 11 patients with choroidal malignant melanoma had a positive response to their aqueous humor on the CAM. Aqueous samples from eyes with an iris and ciliary body malignant melanoma and a metastatic breast carcinoma to the iris had a positive angiogenic response. By contrast, only one of 15 patients undergoing operation for cataracts, glaucoma, or other nonmalignant ocular disease showed an angiogenesis response. The one patient who a positive assay later developed lymphocytic leukemia. These studies show that certain intraocular tumors display angiogenesis capacity before clinically evident neovascular changes of floating tumor cells are seen. Patients without tumors showed no angiogenesis response.

Cyclocryotherapy of chronic open-angle glaucoma in aphakic eyes.

Twenty-six eyes of 25 patients with chronic open-angle glaucoma and surgical aphakia with inadequate control of intraocular pressure (IOP) despite maximal medical treatment were treated with cyclocryotherapy, according to a prospective protocol and specific technique. (Neovascular and synechial glaucoma were excluded from this series.) In most cases topical medications were resumed after cyclocryotherapy. During follow-up of seven to 95 months (average, 46 months), IOP was reduced to 19 mm Hg or lower in 24 out of 26 eyes (92%). In two eyes, persistent decrease in visual acuity, unrelated to reduction of IOP, occurred after cyclocryotherapy. On the basis of the long-term benefits obtained by this procedure, we concluded that cyclocryotherapy should be considered the procedure of choice in the treatment of primary open-angle glaucoma in surgically aphakic eyes when pressure cannot be adequately reduced by maximally tolerated medical treatment.

Effect of cataract surgery on intraocular pressure reduction obtained with laser trabeculoplasty.

Thirty-nine eyes of 34 patients with open-angle glaucoma who had been treated successfully with argon laser trabeculoplasty underwent either extracapsular cataract extraction with posterior chamber intraocular lens implantation or intracapsular cataract extraction without lens implantation. Cataract surgery did not have a significant effect on the magnitude of intraocular pressure reduction obtained with laser trabeculoplasty. This study supports the view that eyes with coexisting cataract and glaucoma may benefit from laser trabeculoplasty before cataract surgery.

Ocular manifestations of juvenile rheumatoid arthritis.

We followed 210 cases of juvenile rheumatoid arthritis closely for eleven years. Thirty-six of the 210 patients (17.2%) developed iridocyclitis. Iridocyclitis was seen most frequently in young female patients (0 to 4 years) with the monoarticular or pauciatricular form of the arthritis. However, 30% of the patients developed uveitis after 16 years of age. Although 61% of patients had a noncontributory ocular history on entry, 42% had active uveitis on entry. Our approach was effective in detecting uveitis in new cases and exacerbations of uveitis in established cases. Forty-four percent of patients with uveitis had one or more identifiable signs or symptoms, such as red eye, ocular pain, decreased visual acuity, or photophobia, in order of decreasing frequency. Even after early detection and prompt treatment, 41% of cases of uveitis did not respond to more than six months of intensive topical treatment with corticosteroids and mydriatics. Despite this, there was a dramatic decrease in the 50% incidence of blinding complications of uveitis cited in earlier studies. Cataract and band keratopathy occurred in only 22 and 13% of our group, respectively. We used chloroquine or hydroxychloroquine in 173 of 210 cases and found only one case of chorioretinopathy attributable to these drugs. Systemically administered corticosteroids were used in 75 of 210 cases; a significant number of posterior subcapsular cataracts was found. Typical keratoconjunctivitis sicca developed in three of the uveitis cases. This association with uveitis and JRA was not noted previously. Surgical treatment of cataracts, band keratopathy, and glaucoma achieved uniformly discouraging results.

Scanning electron microscopy of trabeculectomy specimens in open-angle glaucoma.

We examined trabeculectomy specimens obtained at the time of surgery for inadequately controlled open-angle glaucoma by scanning electron microscopy. Of the 50 specimens ten included a broad area of the trabecular meshwork. In these ten specimens the meshwork was obscured by material. We believe the degree of occlusion was significant in these specimens, which would lead to obstruction of aqueous outflow. No similar material was found in control specimens from persons of similar age.

Argon laser gonioplasty in the treatment of angle-closure glaucoma.

We used argon laser gonioplasty to treat angle-closure glaucoma unrelieved by patent iridectomy. Laser energy (mean; 30 spots, 723 mW, and 0.2 second) was applied to the peripheral iris stroma to open the anterior chamber angle. Twenty of 32 eyes were successfully treated. After a median follow-up period of 18 months, 17 of these 20 successfully treated eyes (85%) had an intraocular pressure less than or equal to 19 mm Hg, and 19 of these 20 successfully treated eyes (95%) had an intraocular pressure less than or equal to 21 mm Hg. The 20 successfully treated eyes had a median duration of angle closure of 12 days. Twelve unsuccessfully treated eyes had a median duration of angle closure of 90 days. All successfully treated eyes had more than 50% of the treated angle opened by argon laser gonioplasty and all but three successfully treated eyes had more than three clock hours opened by argon laser gonioplasty. Argon laser gonioplasty may be successful in treating angle-closure glaucoma unrelieved by iridectomy, especially in cases that are recognized and treated soon after onset.

Uveal melanoma: case report of extension through the optic nerve to the surgical margin in the orbital apex.

An unsuspected uveal melanoma arising in a phthisical eye extended through the optic nerve to the surgical margin in the orbital apex without involvement of other orbital structures. Step sections showed the continuous extension through the nerve. Factors likely to predispose to optic nerve extension include epithelioid cell type, large tumour size, peripapillary involvement, and glaucoma. Optic nerve extension carries the risks noted with orbital extension of intraocular tumour, but may in addition lead more directly to intracranial invasion, including a chiasmal syndrome. The role of exenteration and radiotherapy in these cases remains uncertain.

Cyclocryotherapy for glaucoma.

CCT is a simple, noninvasive, short, ablative surgical procedure that has been effectively used to treat advanced uncontrolled glaucoma. A specific protocol is recommended and the rationale for strict adherence is presented. The long-term benefits of CCT in patients with open-angle glaucoma following cataract extraction have been well established. However, although patients with neovascular glaucoma may have adequate pressure control following CCT, the serious complications of progression to no light perception and phthisis bulbi have discouraged its use. CCT can be effective for neovascular glaucoma when other methods of surgical management prove unsuccessful and visual prognosis is very poor. The loss of functional vision occurred in fewer than 10 percent of eyes treated with CCT, and it is important to emphasize that only eyes with greatest risk and poorest prognosis were treated with this modality. CCT has become an important addition to the surgical armamentarium for the treatment of some specific types of glaucoma.

Bleb infections: clinically different courses of "blebitis" and endophthalmitis.

BACKGROUND AND OBJECTIVES: To assess the differences in history, clinical course, and response between five cases of blebitis and three cases of endophthalmitis following mitomycin trabeculectomy. PATIENTS AND METHODS: The authors conducted a retrospective review of eight consecutive cases of bleb-related infection following successful mitomycin trabeculectomy. RESULTS: All patients with blebitis responded to treatment with return of visual acuity and intraocular pressure to preinfection levels. In the three cases of endophthalmitis, one patient underwent enucleation, one had a final visual acuity of counting fingers, and the third had a visual acuity of 20/60. CONCLUSIONS: Blebitis, a limited form of bleb-related infection with thin, cystic, leaky blebs, responds to intensive topical antibiotic treatment, returning visual acuity and IOP to preinfection levels. Bleb-related endophthalmitis causes a more virulent form of bleb-related infection that involves thin- or thick-walled blebs, with or without leakage, and poor visual prognosis despite immediate intensive topical, systemic, and intravitreal antibiotic administration combined with core vitrectomy.

Open-angle glaucoma secondary to blood clot in the Schlemm's canal following scleral buckle surgery and its treatment with tPA.

Obstruction of the episceral venous system can present with glaucoma. We present two patients who were referred to us for management of acute glaucoma with presumed episceral venous compression. The first patient had open-angle glaucoma with probable elevated episcleral venous pressure and blood in the Schlemm's canal, following a 360 degrees scleral buckle surgery. The second patient had open-angle glaucoma from delayed peribulbar hemorrhage following uncomplicated cataract surgery with blood in the Schlemm's canal. Both patients were successfully treated with intracameral injection with tissue plasminogen activator (tPA).