Sacral nerve stimulation for constipation and fecal incontinence in children: Long-term outcomes, patient benefit, and parent satisfaction.

OBJECTIVE: To evaluate the long-term efficacy of sacral nerve stimulation (SNS) in children with constipation and describe patient benefit and parent satisfaction. METHODS: Using a prospective patient registry, we identified patients <21 years old with constipation treated with SNS for >2 years. We compared symptoms, medical treatment, PedsQL Gastrointestinal Symptom Scale (GSS), Fecal Incontinence Quality of Life Scale (FIQL), and Fecal Incontinence Severity Index (FISI) before SNS and at follow-up. We contacted parents to administer the Glasgow Children's Benefit Inventory (GCBI) and a parent satisfaction questionnaire. KEY RESULTS: We included 25 children (52% male, median age 10 years): 16 had functional constipation, six anorectal malformation, two tethered spinal cord, and one Hirschsprung's disease. Defecation frequency did not change after SNS but patients reporting fecal incontinence decreased from 72% to 20% (P<.01) and urinary incontinence decreased from 56% to 28% (P=.04). Patients using laxatives decreased from 64% to 44% (ns) and patients using antegrade enemas decreased from 48% to 20% (P=.03). GSS, most FIQL domains, and FISI were improved at follow-up. Six (24%) patients had complications requiring further surgery. Of the 16 parents contacted, 15 (94%) parents indicated positive health-related benefit and all would recommend SNS to other families. CONCLUSIONS & INFERENCES: Sacral nerve stimulation is a promising and durable treatment for children with refractory constipation, and appears particularly effective in decreasing fecal incontinence. Although a quarter of patients experienced complications requiring additional surgery, nearly all parents reported health-related benefit. Future studies to identify predictors of treatment response and complications are needed.

Comparison of the impact on quality of life of immediate versus delayed implant placement supporting immediately loaded mandibular overdenture.

OBJECTIVE: No controlled clinical study has evaluated Oral Health Related Quality of Life (OHRQOL) of immediately placed and loaded implants retaining mandibular overdentures. This pilot study evaluated the impact of immediate loading of delayed versus immediately placed dental implants on overdenture patients' OHRQOL. MATERIALS AND METHODS: Two patient treatment groups received a maxillary conventional complete denture, opposing a mandibular overdenture immediately loaded on implants with ball attachments; In group one a delayed implant placement surgical protocol was followed, whereas in group two, patients had mandibular teeth extracted and two implants immediately placed. OHRQOL for these patients was evaluated using a visual analog scale for 48 questions related to 6 domains; comfort, function, speech, esthetics, self-image and dental health. RESULTS: There was no significant difference between the two groups when comparing answers to the pre- and post-treatment questionnaires. Pooled data showed significant differences for all domains between the pre-treatment and post-treatment responses, denoting improvement after an implant retained mandibular overdenture was delivered. CONCLUSION: There was an improved quality of life in patients receiving immediately loaded mandibular overdentures supported by either delayed or immediately placed implants.

Students' perception of peer-assessment in the context of a treatment planning course.

OBJECTIVE: Peer assessment is grounded in philosophies of active learning, and it would seem that this tool is a viable method for critical thinking development. The purpose of this article was to present how junior students at Case Western Reserve University School of Dental Medicine (CWRU) perceive the value of a peer-assessment activity in the context of a treatment planning course. METHODOLOGY: As a part of the final exam for the junior year Treatment Planning course, students were requested to evaluate a de-identified assignment submitted by one of their peers. Following the exam, a survey was sent to the students to determine how they perceived the peer-assessment activity and how this relates to other learning experiences in the course. RESULTS: Our results show that students' perception of the benefit of peer grading was not associated with any individual peer-assignment characteristics, or course characteristics. Similar results were obtained regarding the perceived benefit of identifying evidence. Moderate correlations were observed between peer evaluation characteristics. CONCLUSIONS: It was concluded that: (i) junior dental students are not homogenous in their opinions regarding the value of an activity related to evaluation of a peer's assignment and (ii) student's perceptions regarding the peer-grading component of peer assessment were not correlated with perceptions related to other learning processes in the treatment planning course.

[MRI of the temporomandibular joint--the gold standard].

MRI has been established as the imaging modality of choice for the evaluation of temporomandibular joint disorders (TMD), as it allows for a noninvasive detailed evaluation of the joint that is not otherwise available. During the last decade, the introduction of dynamic (cine-mode) MR imaging has made functional evaluation feasible in addition to the morphologic study of the joint. The major advantage of MRI is its ability to study the articular disc and its congruity as well as its location relative to the condyle in both closed- and open-mouth positions. Due to its high contrast resolution, MRI is unique in demonstrating joint effusion, bone edema and sclerosis, rupture of the retrodiscal layers and impairment of the lateral pterigoid muscle. In Mor-Mar Imaging Center we performed during the last two years MRI studies of the TMJ in 234 subjects, 172 (74%) female and 62 (26%) male patients. The average age of the patients was 29 years. In this article we present our experience in the evaluation of TMD and review the main indications and findings. In addition, our experience in optimizing the MRI sequence protocols in both static and dynamic modes are discussed. MRI examinations provide the clinician with anatomic and physiologic information that can guide treatment decisions. It has a special role in monitoring and evaluating treatment results, both conservative and surgical.

Improvement of quality of life and symptoms after gastric electrical stimulation in children with functional dyspepsia.

BACKGROUND: Our objective is to evaluate the effect of gastric electrical stimulation (GES) on symptoms and quality of life for pediatric patients with functional dyspepsia (FD). METHODS: Twenty-four patients (16 female, median 15 years) were treated with GES for FD after a median of 24 months of symptoms (3 months-14 years). At baseline, 46% required tube feeds and 25% parenteral nutrition. Sixty percent had gastroparesis. The PedsQL GI Module (PedsQL) was completed for 18/24 both pre-/post-GES after a median of 8 months. Patients also completed the Symptom Monitor Worksheet (SMW) pre-/post-GES after a median of 6 months. Pre-/post-GES global health was also assessed. KEY RESULTS: Significant improvements were seen in multiple areas of the PedsQL, including stomach pain/upset, food/drink limits, heartburn/reflux, gas/bloating, patient worry, medication tolerance, and constipation (P < 0.05). A decrease was found in combined symptom severity/frequency based on SMW (P < 0.01). Improvements were made in all categories, including vomiting, nausea, early satiety, bloating, fullness, epigastric pain, and burning (P < 0.01). Improvements in PedsQL/SMW scores remained when analysis was limited to normal or delayed gastric emptying (P < 0.05, P < 0.05). Thirteen percent needed tube feeds and 13% parenteral nutrition after GES. Sixty-five percent reported that their health was much improved after GES vs 15% the same or worse. Five patients experienced complications, primarily mild abdominal discomfort. CONCLUSIONS & INFERENCES: In the largest series to date of pediatric patients who have undergone GES for FD, we found significant improvements in upper gastrointestinal symptoms, quality of life, and perception of global health. Patients were less dependent on tube feeding or parenteral nutrition.

Students' satisfaction with electrical handpieces in an educational setting.

OBJECTIVE: This article presents the evaluation and outcome of improvements carried out at Case Western Reserve School of Dental Medicine (CWRU) based on feedback provided by students during implementation of electrical handpieces technology in the school. METHODOLOGY: Students were surveyed in February 2010 (first survey) regarding their satisfaction with the existing clinical set-up for electrical handpieces. Following a change in clinical setting and integration of the control box into the dental unit in November 2010, students were administered the same survey as in February 2010 (second survey). RESULTS: There was an increased level of satisfaction with electric handpieces in the new clinical setting; these levels were significant regarding the ergonomics of the handpiece and clinical setting, operation of the handpiece, and technical maintenance. There was a significant shift from those who were categorically against using electrical handpieces after graduation towards those were 'not sure' regarding the adoption of electric technology in their practice. CONCLUSIONS: Specifically improving the clinical setting of a control box for electrical handpieces can influence overall student perception regarding the quality of handpieces and their operation.

Early metabolic improvement following bariatric surgery in morbidly obese adolescents.

Bariatric surgery results in durable weight loss and improved comorbidities. The objectives of this study were to examine the efficacy of gastric bypass in reducing comorbid burden and improving metabolic status among morbidly obese adolescents. The medical records of 15 gastric bypass patients were retrospectively reviewed. Changes in metabolic markers were determined at baseline, 1 and 2 years post-operatively. Comparative analysis demonstrated significant improvement in weight, BMI, insulin, HbA1C, C-peptide, %B, %S, IR, cholesterol, percentile cholesterol, TG, percentile TG, HDL, percentile HDL, LDL, percentile LDL, and VLDL. Results support bariatric surgery as a treatment for morbidly obese adolescents with comorbidities.

Streamlining the care of patients with hypertrophic pyloric stenosis: application of a clinical pathway.

PURPOSE: The purpose of this study was to evaluate the impact of a clinical pathway on infants admitted to a pediatric tertiary care center with the diagnosis of hypertrophic pyloric stenosis (HPS). METHODS: The records of 132 HPS patients were evaluated before and after implementation of a clinical pathway. Infants were excluded for prematurity, admission to nonsurgical services, or multiple diagnoses requiring prolonged hospitalization, resulting in 83 patients for analysis. Group I (prepathway, n = 40) and group II (postpathway, n = 43) infants were analyzed for time from admission to operation, operation to first feeding, operation to discharge, total length of stay, hospital charges, metabolic status at time of admission, and postoperative complications. The Mann-Whitney test was performed (statistical significance at P <.05). RESULTS: There was no significant difference between group I and group II patients in the length of preoperative hospitalization or metabolic status at the time of hospital admission. In comparison with group I patients, there was a significant reduction in time to resumption of oral feedings (4.6 +/- 1.9 hours v 7.5 +/- 3.2 hours; P <.001) for group II infants and a significantly earlier discharge (26.7 +/- 6.8 hours v 38.0 +/- 11.7 hours; P <.001). This resulted in a shortened length of stay (41.8 +/- 9.7 hours v 57.8 +/- 14.3 hours; P <.001) with an associated decrease in hospital charges ($4,555 +/- $464 v $5,400 +/- $1,017; P <.001). CONCLUSIONS: Elimination of practice variability by the use of a clinical pathway for HPS resulted in significant reduction of hospital stay and related charges. The impact of the pathway occurred in the postoperative period and is a consequence of a rapid and systematic return to oral feedings.

Preoperative diagnosis of extralobar pulmonary sequestration with unusual vasculature: a case report.

Pulmonary sequestrations are uncommon congenital malformations of the lung. Vascular supply to pulmonary sequestrations is variable, with many unusual combinations. Adequate preoperative evaluation is hence advisable to avoid intraoperative complications. Magnetic resonance imaging (MRI) allows accurate diagnosis of extralobar pulmonary sequestration (ELPS), including definition of vascular supply. This may obviate the need for more invasive investigations that were suggested in the past. Presented here is a case of ELPS with its vascular attachments to the intercostal vessels.

The duodenal dimple: a specific fluoroscopic correlate to the duodenal web.

PURPOSE: We describe a new fluoroscopic sign to aid in the diagnosis of an obstructing duodenal web and its attachment site. MATERIALS AND METHODS: During an upper GI series of a neonate, a nasogastric tube was passed into an obstructed duodenum and barium injected. The tube, pressing on an obstructing web, caused dimpling of the duodenal contour at the attachment point of the web to the duodenal wall. The same maneuver at surgery caused identical dimpling. DISCUSSION: While the maneuver is described in surgical textbooks, there has been no radiologic correlate. The "duodenal dimple" is a new fluoroscopic sign of a duodenal web and its attachment point to the duodenal wall.

Cytomegalovirus infection in patients with HIV infection.

Cytomegalovirus (CMV) is responsible for the most common viral opportunistic infection in persons with acquired immunodeficiency virus syndrome (AIDS). Clinical disease due to CMV has been recognized in up to 40% of patients with advanced HIV disease. The most common presentation is retinitis, although colitis, esophagitis, pneumonitis and neurological disorders are also reported frequently. CMV retinitis is usually diagnosed clinically, and serological testing for CMV immunoglobulin is useful to support the diagnosis. Parts of the gastrointestinal tract (esophagus and colon) are the most common extraocular sites of CMV infection in AIDS patients. Therapy with systemic agents, including intravenous ganciclovir, intravenous foscarnet, and intravenous cidofovir, is effective. Ganciclovir is associated mainly with hematological toxicity, while foscarnet and cidofovir are nephrotoxic. Intravitreal injections with these antiviral agents are also effective, but inconvenient, and there is a need for repeated injections. Intraocular implants that slowly release ganciclovir have been effective for both acute therapy and long-term maintenance, but the occurrence of contralateral ocular and extraocular disease is a serious concern. New agents, as for example an anti-sense agent against CMV, appear promising.

Primary intrathoracic gastric volvulus in a newborn.

Primary gastric volvulus in the neonatal period is extremely rare, with only 18 cases reported in the literature. The authors describe the case of a newborn with intrathoracic gastric volvulus and discuss its management.

Periumbilical pseudoxanthoma elasticum associated with chronic renal failure and angioid streaks--apparent regression with hemodialysis.

Pseudoxanthoma elasticum (PXE) is a heritable connective tissue disease involving progressive fragmentation and dystrophic calcification of elastic fibers. Periumbilical disease as the exclusive site of cutaneous involvement is most commonly seen in the rare entity termed periumbilical perforating pseudoxanthoma elasticum (PPPXE). Patients with this disorder are generally obese, middle aged, multiparous black women with hypertension. The cutaneous lesions are well-demarcated, hyperpigmented, periumbilical plaques with keratotic papules on the periphery. Extracutaneous manifestations have rarely been described. We describe a patient with periumbilical PXE associated with chronic renal failure and bilateral angioid streaks. Histopathologic examination demonstrated typical calcification of elastic fibers with additional amorphous calcium deposits in the superficial dermis. Transepidermal elimination was not present. Normalization of the serum calcium-phosphate product resulted in regression of the lesions--both clinically and histopathologically. The relation between PPPXE and hereditary PXE is discussed. The role of chronic renal failure in precipitating PPPXE is considered.

Prognostic classification for esophageal atresia and tracheoesophageal fistula: Waterston versus Montreal.

Since 1962, the Waterston classification has been used to stratify neonates who have esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) into prognostic categories based on birth weight, the presence of pneumonia, and the identification of other congenital anomalies. In response to advances in neonatal care, the surgeons from the Montreal Children's Hospital proposed a new categorization system in 1993 in an attempt to define the current risk factors for patients who have EA/TEF. In the Montreal experience only two characteristics independently affected survival: preoperative ventilator dependence and associated major anomalies. The goal of this study was to determine which system had the greatest validity for the evaluation of prognosis in our patients with EA/TEF. The charts of 94 patients who had EA/TEF treated between 1972 and 1991 were reviewed. Patients were classified using both the Waterston and Montreal systems. Groups were compared with Fisher's Exact test using a 95% confidence level for statistical significance. Eleven infants were ventilator dependent preoperatively; 62 children had major associated anomalies, 8 of which were considered life threatening. Sixteen children died within 4 years, eight during their initial hospital stay. Five of the eight early postoperative deaths occurred in the highest-risk patients (Waterston C or Montreal II). Analysis was performed for multiple risk factors and mortality. As in the Montreal study, the presence of life-threatening and major congenital anomalies represented significant risk factors for death. Pulmonary disease as delineated by ventilator dependence appeared to be more accurate than pneumonia. This study confirms the accuracy of the Montreal classification in defining prognosis for EA/TEF. The Montreal system more accurately identifies children at highest risk than the Waterston classification.

Management of varicella zoster virus retinitis in AIDS.

AIMS/BACKGROUND: Varicella zoster virus retinitis (VZVR) in patients with AIDS, also called progressive outer retinal necrosis (PORN), is a necrotising viral retinitis which has resulted in blindness in most patients. The purposes of this study were to investigate the clinical course and visual outcome, and to determine if the choice of a systemic antiviral therapy affected the final visual outcome in patients with VZVR and AIDS. METHODS: A review of the clinical records of 20 patients with VZVR from six centres was performed. Analysis of the clinical characteristics at presentation was performed. Kruskall-Wallis non-parametric one way analysis of variance (KWAOV) of the final visual acuities of patients treated with acyclovir, ganciclovir, foscarnet, or a combination of foscarnet and ganciclovir was carried out. RESULTS: Median follow up was 6 months (range 1.3-26 months). On presentation, 14 of 20 patients (70%) had bilateral disease, and 75% (15 of 20 patients) had previous or concurrent extraocular manifestations of VZV infection. Median initial and final visual acuities were 20/40 and hand movements, respectively. Of 39 eyes involved, 19 eyes (49%) were no light perception at last follow up; 27 eyes (69%) developed rhegmatogenous retinal detachments. Patients treated with combination ganciclovir and foscarnet therapy or ganciclovir alone had significantly better final visual acuity than those treated with either acyclovir or foscarnet (KWAOV: p = 0.0051). CONCLUSIONS: This study represents the second largest series, the longest follow up, and the first analysis of visual outcomes based on medical therapy for AIDS patients with VZVR. Aggressive medical treatment with appropriate systemic antivirals may improve long term visual outcome in patients with VZVR. Acyclovir appears to be relatively ineffective in treating this disease.

Evaluation of a new self-screening chart for cytomegalovirus retinitis in patients with AIDS.

AIDS patients often present without visual symptoms despite severe vision-threatening cytomegalovirus (CMV) retinitis. A new self-examination chart, the Teich Target (copyright 1993, Steven A. Teich, pat. no. 370259), which tests the central 45 degrees of vision, was evaluated for its ability to screen AIDS patients for CMV retinitis. It was compared with the Amsler grid (which tests the central 20 degrees of vision) in 50 consecutive AIDS patients referred for ophthalmologic evaluation by a single practitioner. Of 19 patients found to have CMV retinitis, 12 (63%) noted scotomas with the Teich Target compared with 7 (37%) with the Amsler grid (p < 0.05). Overall, 13 of 20 (65%) infectious retinitis cases were detected by the Teich Target. and 7 of 20 (35%) with the Amsler grid (p < 0.05). All scotomas noted on the Amsler grid were detected by the Teich Target. Four visually asymptomatic patients with newly diagnosed CMV retinitis noted scotomas only with the Teich Target. Both methods detected a branch retinal artery occlusion, but only the Teich Target detected a quadrantanopsia due to cerebral toxoplasmosis. There were no false positives with either method. In this small series, the Teich Target was more effective than the Amsler grid in screening AIDS patients for CMV retinitis primarily because it detected lesions beyond the central 20 degrees of vision. Peripheral CMV retinitis beyond the central 40 degrees occurred in 37% of patients and was net detected by either method. The Teich Target therefore augments but does not replace periodic ophthalmologic evaluations. The Teich Target may have potential utility for home use as a patient self-screening test for CMV retinitis or for mass screening in an AIDS clinic.

Dicephalus dipus dibrachius: an unusual case of conjoined twins.

Conjoined twinning is a rare anomaly, occurring in one of every 50,000 births. Dicephalus dipus dibrachius is an extremely rare form of conjoined twinning in which the infant has two arms, two legs, one trunk, but two heads. These infants are often stillborn or die shortly after birth. The authors describe a case of dicephalus twinning with 11-day survival. A thorough investigation using multiple imaging modalities (plain radiographs, contrast studies, ultrasonography, and magnetic resonance imaging) demonstrated that these twins had separate spinal columns but shared multiple internal organs including heart, liver, pancreas, intestine and bladder. Based on the anatomy of this case, the authors conclude that separation of dicephalus dipus dibrachius twins should not be attempted.

Successful treatment of progressive outer retinal necrosis syndrome.

PURPOSE: Progressive outer retinal necrosis is a destructive retinopathy found in patients with acquired immune deficiency syndrome. Treatment of this disorder has been unsuccessful in reported patient series, with the patients experiencing profound bilateral loss of vision. METHODS: We treated six patients with combination antiviral therapy, usually with intravenous foscarnet and either ganciclovir or acyclovir. RESULTS: These six patients retained a visual acuity of 20/100 or better in at least one eye for the remainder of their lives (a period > 4 months for each patient). Retinal detachments developed in four patients, for which vitrectomy and silicone oil tamponade were required. CONCLUSIONS: A combination of intravenous antiviral therapy and aggressive vitrectomy techniques to repair any associated detachments may allow the preservation of useful visual acuity in patients with progressive outer retinal necrosis. This is the first reported series of successful long-term treatment of patients with this disorder.

Successful management of a chylothorax in infancy using a pleurectomy.

The management of chylothorax is described in three infants. Because none of them had had a direct injury to the thoracic duct, it was suspected that the chylothoraces had a significant chance of recurrence after simple ligation of the duct. To avoid recurrence, formal stripping of the parietal pleura was performed using a technique not previously reported in the literature. The operation was simple to perform and resulted in resolution of the chylothorax in all infants. The three cases and details of the surgical procedure are described.

High-dose-rate brachytherapy in childhood sarcomas: a local control strategy preserving bone growth and function.

The administration of external beam radiation therapy (EBRT) has been an integral part of the successful treatment of childhood sarcomas. However, EBRT has severe late morbidity in the developing child. In an attempt to deliver adequate tumoricidal radiation while preserving bone growth and organ function, 13 children with diverse sarcomas were treated with high dose rate brachytherapy (HDR). Seven patients had rhabdomyosarcoma and six patients had other soft tissue sarcoma variants. All patients were treated with disease-appropriate chemotherapy, usually according to the intergroup Rhabdomyosarcoma Study. Eleven patients received fractionated 36 Gy HDR alone at a mean of 3.5 months from diagnosis. Two patients received 10-12.5 Gy intraoperative HDR brachytherapy and additional 27 Gy EBRT. Nine of 11 patients in first remission have had no recurrences. One died of recurrent pulmonary metastases. The other patient that did recur is disease-free 21 months post-recurrence. Two additional patients were treated with HDR after tumor recurrence. One patient with recurrent Ewing's sarcoma, relapsed and died. The second is disease free 3 months after autologous bone marrow transplant. Grade 1 morbidity occurred in 46%, Grade 2 in 15%, and Grade 3 in 8% of the children, while relatively good bone and organ growth was maintained. The combination of conservative surgery, chemotherapy, and HDR offers the potential for disease control in young children while preserving bone growth and organ function.