Ketamina frente a midazolam como adyuvante para el bloqueo peribulbar utilizando una única inyección inferonasal en los pacientes sometidos a vitrectomía: ensayo controlado aleatorizado / Ketamine versus midazolam as an adjuvant to peribulbar block using a single inferonasal injection in patients undergoing vitreoretinal surgery: A randomized controlled trial

Introducción y objetivos: El objetivo de este estudio fue evaluar la seguridad y eficacia de midazolam y ketamina como adyuvantes para el bloqueo peribulbar en vitrectomías. Pacientes y métodos: Este ensayo controlado aleatorizado incluyó 93 pacientes adultos sometidos a vitrectomías realizadas con anestesia peribulbar. Se asignó aleatoriamente a los pacientes en 3 grupos (de 31 participantes cada uno): control (mezcla anestésica estándar), midazolam (mezcla estándar+midazolam) y ketamina (mezcla estándar+ketamina). Los resultados primarios fueron el inicio de la acinesia globular y la duración de la analgesia. Los resultados secundarios fueron la duración del bloqueo motor, el inicio de la anestesia corneal y la acinesia palpebral, y los cambios de los datos vitales (presión sanguínea, saturación de oxígeno, y frecuencia cardiaca). Resultados: El grupo ketamina frente al grupo control y el grupo midazolam reflejaron el inicio más rápido de la acinesia palpebral y globular (p<0,001) y la anestesia corneal (0,7±0,2 vs. 1,5±0,5 y 1,2±0,4, respectivamente; p<0,001) y la duración más prolongada tanto de la analgesia (3,7±0,6 vs. 2,3±0,4 y 3,1±0,6, respectivamente; p<0,001) como de la acinesia (3,8±0,5 vs. 3±0,4, y 3,7±0,5, respectivamente; p<0,001). El grupo midazolam reflejó mejores resultados que los controles, aunque el fármaco fue menos efectivo que la ketamina. No se produjeron diferencias significativas en términos de datos vitales entre los grupos (p>0,05). Conclusiones: Ketamina es un adyuvante efectivo para el bloqueo peribulbar. Mejora el bloqueo motor y sensorial acelerando el inicio y prolongando la duración. Dichos efectos son deseables en los procedimientos oftálmicos más prolongados, tales como vitrectomías. Los efectos de ketamina fueron superiores a los de midazolam.(AU)

Introduction and objectives: This study aimed to assess the safety and efficacy of midazolam and ketamine as adjuvants to the peribulbar block in vitreoretinal surgeries. Patients and methods: This randomized controlled trial included 93 adult patients undergoing vitreoretinal surgeries performed with peribulbar anaesthesia. Patients were randomly allocated to 3 groups (31 participants each): control (standard anaesthetic mixture), midazolam (standard mixture+midazolam), and ketamine (standard mixture+ketamine). The primary outcomes were onset of globe akinesia and duration of analgesia. Secondary outcomes were duration of motor blockade, onset of corneal anaesthesia and lid akinesia, and changes in vital data (blood pressure, oxygen saturation, and pulse rate). Results: The ketamine group vs. the control and midazolam groups showed the most rapid onset of lid and globe akinesia (p<0.001) and corneal anaesthesia (0.7±0.2 vs. 1.5±0.5 and 1.2±0.4, respectively; p<0.001) and the longest duration of both analgesia (3.7±0.6 vs. 2.3±0.4 and 3.1±0.6, respectively; p<0.001) and akinesia (3.8±0.5 vs. 3.0±0.4, and 3.7±0.5, respectively; p<0.001). The midazolam group showed better outcomes than controls, but the drug was less effective than ketamine. There were no significant differences in vital data among groups (p>0.05). Conclusions. Ketamine is an effective adjuvant for peribulbar blockade. It enhances both motor and sensory blockade by hastening onset and prolonging duration. These effects are desirable in lengthier ophthalmic procedures such as vitreoretinal surgeries. The effects of ketamine were superior to those of midazolam.(AU)

LICAP Versus TDAP for the Reconstruction of Partial Breast Defects.

Perforator flaps are the latest development in reconstructive surgery. Pedicled chest wall perforator flaps can be utilized in many cases of partial breast reconstruction. This research compares the outcome and technique of thoracodorsal artery perforator flap (TDAP) and the lateral intercostal artery perforator flap (LICAP) in the reconstruction of partial breast defects. Patient records were reviewed for the time period between 2011 and 2019 at the Breast Unit of the National Cancer Institute of Cairo University. Eighty three patients were accessible for the study. (46 cases of TDAP flap and 37 cases of LICAP flap). Relevant clinical data were extracted from patients' records. A special visit was organized for all 83 patients, where a digital photograph was taken in an antroposterior view. The photographs were later processed via BCCT.core software to obtain an objective cosmetic outcome assessment. Complication rates and cosmetic outcome were comparable for both techniques. TDAP flap proved to require more tedious dissection and preoperative Doppler mapping to localize perforator vessels. On the other hand, LICAP was technically easier with more consistent perforators. Pedicled chest wall perforator flaps constitute an excellent reconstructive option in partial breast defects. TDAP flap and LICAP are two reliable perforator flaps which can reconstruct outer breast defects with acceptable outcome.

Screening and Risk Factors for Retinopathy of Prematurity in a Tertiary Care Hospital in Cairo, Egypt.

Purpose: To evaluate the retinopathy of prematurity (ROP) prevalence, risk factors and screening outcome in a tertiary hospital in Cairo, Egypt. Methods: A prospective observational study was done in Neonatal Intensive Care Unit in Ain Shams University Hospital. A total of 159 premature infants were screened for ROP based on the most inclusive criteria reported to date. Screening included premature infants with gestational age (GA) of ≤34 weeks or birth weight (BW) of ≤2000 grams, or GA >34 weeks or BW >2000 grams, with multiple co-morbidities. The prevalence of ROP, plus disease and their correlation with risk factors of interest were studied. Results: The GA of the included infants ranged from 27 to 36 weeks, mean (SD) 31.87 (± 1.81) weeks. The BW ranged from 640 to 3900 grams, mean (SD) 1784.71 (± 560.30) grams. The prevalence of ROP more than stage 0 was 25.8% (41 infants), 7.3% of the cases (11 infants) showed plus disease and 6.3% (10 infants) showed severe ROP requiring treatment. Of those, 2 cases (20%) fell outside the British Guideline's criteria for Screening. There was a highly significant (p < 0.0001) correlation between ROP more than stage 0 and low GA, low BW, mechanical ventilation, respiratory distress syndrome, necrotizing enterocolitis, intraventricular haemorrhage, and blood transfusion. No significant correlation was found between appearance of ROP more than stage 0 and gender (p = 0.911), patent ductus arteriosus (p =0.187), or sepsis (p =0.998). Conclusion: ROP is a significant problem in the premature infants in Egypt. Extremely premature infants with lower BW are more prone to develop ROP. However, cases with higher GA and BW than mentioned in the British guidelines screening criteria especially with multiple comorbidities showed severe ROP requiring intervention, which implies the need to develop a screening guideline for the Egyptian population.

The dilemma after an unforeseen aortic arch anomalies at thoracoscopic repair of esophageal atresia: Is curtailing surgery still a necessity?

BACKGROUND AND OBJECTIVE: There are several forms of relevant epi-aortic branching anomalies, and perhaps that is why different views as to the best approach have been reported. To help resolve this dilemma, we examined the unforeseen arch anomalies found at thoracoscopic repair of esophageal atresia and the outcomes. METHODS: In a retrospective cohort, all consecutive patients who were thoracoscopically approached for esophageal atresia over a 5-year period with unforeseen aortic/epi-aortic branching were identified and grouped. Thoracoscopic views, operative interventions, and outcomes were studied. RESULTS: A total of 121 neonates were thoracoscopically approached for EA, of whom 18 cases with aberrant aortic architecture were selected. Four (3%) cases were diagnosed on a preoperative echocardiography as a right-sided aortic arch, whereas unforeseen anomalous anatomies were reported in 14 cases (11.6%): left aortic arch with an aberrant right subclavian artery (ARSA) (n = 10), right-sided aortic arch with an aberrant left subclavian artery (ALSA) (n = 3), and mirror-image right arch (n = 1). Single postoperative mortality was reported among the group with left arch and ARSA (10%), whereas all the cases with right arch and ALSA died. CONCLUSIONS: In all, 11.6% of the studied series exhibited unexpected aberrant aortic architecture, with higher complication rates in comparison to the typical thoracoscopic repairs. For EA with left aortic arch and ARSA, the primary esophageal surgery could safely be completed. Meanwhile, curtailing surgery-after ligating the TEF-to get advanced imaging is still advised for both groups with the right arch due to the significant existence of vascular rings.

Unique presentations of aspergillosis endogenous endophthalmitis in two hospitalized patients with severe COVID-19.

We present two ICU-hospitalized patients with coronavirus disease-19 (COVID-19) presenting with endogenous endophthalmitis in one eye and variable manifestations of chorioretinitis in the fellow eye. Two diabetic patients (57 and 62 years old) showed anterior uveitis and yellowish-white subretinal infiltrations. The fellow eye of one patient showed patches of choroiditis, while the other showed full retinal thickness infiltrations. A workup yielded high serum titers of galactomannan, diagnostic of aspergillosis. The widespread use of high doses of corticosteroids in the management of COVID-19 may predispose to various secondary fungal opportunistic infections and may manifest in different forms of chorioretinal infiltration.

Optimism for the Single-stage Transanal Swenson in Neonates.

BACKGROUND: Hirschsprung's disease (HD) has been traditionally treated from infancy onward and different techniques have been used including Swenson, Soave, and Duhamel procedures. The purpose of this study was to evaluate the transanal Swenson's procedure for classical rectosigmoid HD in neonates. PATIENT AND METHODS: This was a prospective study in which neonates diagnosed with HD were recruited from January 2017 to December 2018. Cases with a transition zone proximal to the midsigmoid were excluded. All patients underwent a transanal Swenson's procedure in the neonatal period using a unified protocol. Intraoperative course and postoperative outcomes such as leak, pelvic abscess, soiling, perianal excoriation, stricture, enterocolitis, and constipation were evaluated and all patients were followed for at least 6 months. RESULTS: Twenty-three patients (17 males and 6 females) underwent transanal Swenson's procedure. The mean age was 22 ± 5.7 days. Follow-up ranged from 6 to 30 months. No anastomotic leak, retraction, or prolapse was reported. Mild perianal excoriations occurred early in 9 (39%) cases and all responded to medical treatment and disappeared before 2 months postoperatively. Stricture occurred in 2 (8%) patients, enterocolitis in 3 (13%), and constipation in 3 (13%). CONCLUSION: Transanal Swenson's procedure is feasible and can be performed safely in neonates with rectosigmoid HD with good short-term outcomes. Proper patient selection and standard protocol following fine procedural hall-marks and details are keys for optimal results and patient satisfaction.

Autologous Retinal Transplantation for Primary and Refractory Macular Holes and Macular Hole Retinal Detachments: The Global Consortium.

PURPOSE: To report the anatomic and functional outcomes of autologous retinal transplantation (ART). DESIGN: Multicenter, retrospective, interventional, consecutive case series. PARTICIPANTS: One hundred thirty eyes of 130 patients undergoing ART for the repair of primary and refractory macular holes (MHs), as well as combined MH-rhegmatogenous retinal detachment (MH-RRD), between January 2017 and December 2019. METHODS: All patients underwent pars plana vitrectomy and ART, with surgeon modification of intraoperative variables. A large array of preoperative, intraoperative, and postoperative data was collected. Two masked reviewers graded OCT images. Multivariate statistical analysis and subgroup analysis were performed. MAIN OUTCOME MEASURES: Macular hole closure rate, visual acuity (VA), external limiting membrane and ellipsoid zone (EZ) band integrity, and alignment of neurosensory layers (ANL) on OCT. RESULTS: One hundred thirty ART surgeries were performed by 33 vitreoretinal surgeons worldwide. Patient demographics were: mean age of 63 ± 6.3 years, 58% female, 41% White, 23% Black, 19% Asian, and 17% Latino. Preoperative VA was 1.37 ± 0.12 logarithm of the minimum angle of resolution (logMAR; Snellen equivalent, approximately 20/500), which improved significantly to 1.05 ± 0.09 logMAR (Snellen equivalent, approximately 20/225; P < 0.001) after surgery (mean follow-up, 8.6 ± 0.8 months). Autologous retinal transplantation was performed for primary MH repair in 27% of patients (n = 35), for refractory MH in 58% of patients (n = 76; mean number of previous surgeries, 1.6 ± 0.2), and for MH-RRD in 15% of patients (n = 19). Mean maximum MH diameter was 1470 ± 160 µm, mean minimum diameter was 840 ± 94 µm, and mean axial length was 24.6 ± 3.2 mm. Overall, 89% of MHs closed (78.5% complete; 10% small eccentric defect), with a 95% closure rate in MH-RRD (68.4% complete; 26.3% small eccentric defect). Visual acuity improved by at least 3 lines in 43% of eyes and by at least 5 lines in 29% of eyes. Reconstitution of the EZ (P = 0.02) and ANL (P = 0.01) on OCT were associated with better final VA. Five cases of ART graft dislocation (3.8%), 5 cases of postoperative retinal detachment (3.8%), and 1 case of endophthalmitis (0.77%) occurred. CONCLUSIONS: In this global experience, patients undergoing ART for large primary and refractory MHs and MH-RRDs achieved good anatomic and functional outcomes, with low complication rates despite complex surgical pathologic features.

DNA barcoding and genetic fidelity assessment of micropropagated Aenhenrya rotundifolia (Blatt.) C.S. Kumar and F.N. Rasm.: a critically endangered jewel orchid.

Aenhenrya rotundifolia is a critically endangered terrestrial jewel orchid. It is monotypic and endemic to evergreen forests of southern western ghats of India. In the present study, identification of this plant species is validated with DNA barcoding using matK and rbcL chloroplast markers. Further, germ-free juvenile axillary bud explants were cultured on Mitra medium supplemented with different kinds of cytokinins like 6-benzyladenine, 6-furfurylaminopurine, N6-(Δ2-isopentyl) adenine, thidiazuron, zeatin and meta-topolin as well as auxins such as α-naphthaleneacetic acid, indole-3-acetic acid and indole-3-butyric acid at different concentrations and combinations for successful proliferation and establishment in vitro. After 12 weeks of culture, axillary bud explants produced an average of 30.12 ± 0.71 shoots per explant, 3.87 ± 0.06 cm shoot length, 1671 ± 2.82 mg fresh mass of proliferated shoots with a proliferation frequency of 100% on Mitra medium supplemented with 6.20 µM meta-topolin and 2.25 µM thidiazuron. No root formation was observed in in vitro proliferated microshoots. However, tiny hair like projections were observed in some elongated shoots on Mitra medium pertaining to 5.37 µM NAA. The tiny hair like structure bearing plantlets were hardened and acclimatized with 100% survival rate in the polytunnel chamber. After 8-10 months of establishment ex vitro, flowering was observed. Additionally, the genetic fidelity of in vitro derived plants was tested with ISSR and SCoT marker profiling. The test results revealed that the plants derived from the protocol has 99% genetic similarity to that of the donor mother plant. This study can be applied in forensic interventions of this species, describes the maintenance of germplasm in vitro and establishment of new viable population in its original habitats by restoring existing sites of this critically endangered jewel orchid.

Prophylactic Effect of Opuntia ficus indica Fruit Peel Extract against Irradiation-Induced Colon Injury in Rats.

Opuntia ficus-indica extract has been used in traditional folk medicine for several purposes and exhibits anti-inflammatory properties. This study was directed to explore the prophylactic effect of O. ficus-indica fruit peel extract against irradiation-induced colitis in rats. GC/MS analysis of the petroleum ether extract led to recognition of 33 compounds in the unsaponifiable fraction and 15 fatty acid methyl esters in the saponifiable part. Thirteen terpenes and sterols were isolated and identified from which ten compounds were not isolated from any part of this species before. Data showed that irradiation induced colon injury as manifested by elevated contents of malondialdehyde, nitric oxide, myeloperoxidase, intercellular adhesion molecule-1, cyclooxygenase-2, tumor necrosis factor alpha, and nuclear factor kappa B, while it reduced superoxide dismutase activity and interleukin 10 content in colonic tissues, which was confirmed by histopathological examination. Pretreatment with O. ficus-indica extract attenuated the alteration in the measured parameters. It could be concluded that O. ficus-indica fruit peel extract can be regarded as a potential agent in limiting colonic complications due to irradiation, possibly by its antioxidant and anti-inflammatory properties.

Optical Coherence Tomography Angiography in Monitoring Proliferative Macular Telangiectasia Type 2 Treatment Response.

BACKGROUND AND OBJECTIVE: Difficulty exists in the follow-up of proliferative macular telangiectasia type 2 (MacTel 2) cases after anti-vascular endothelial growth factor (VEGF) treatment due to staining in fluorescein angiography (FA) and alteration in retinal layers by optical coherence tomography (OCT). Herein, the authors report three cases in which OCT angiography (OCTA) could resolve this issue. PATIENTS AND METHODS: In this retrospective, observational case series, diagnosis of MacTel 2 was made based on clinical examination, FA, OCT, and OCTA at presentation. Regression of neovessels was monitored by OCT and OCTA. RESULTS: OCTA could delineate neovessels before treatment in all cases and facilitate differentiation between active and regressed lesions after treatment. Simultaneous OCT images were less easily appreciated due to altered retinal structure secondary to degenerative nature of the disease. CONCLUSION: OCTA could be the tool of choice to monitor neovascular response to anti-VEGF treatment in proliferative MacTel 2. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:485-491.].

Does overbite reduction affect smile esthetics?

OBJECTIVES: To compare the effects of two common methods of overbite reduction on smile esthetics. MATERIALS AND METHODS: A prospective clinical trial was conducted with 32 patients in whom overbite reduction was achieved using a maxillary incisor intrusion arch (18 patients) or flat anterior bite plate (14 patients). Clinical and cephalometric records were compared pretreatment (T1), after overbite reduction (T2), and posttreatment (T3). RESULTS: Both treatment groups experienced a reduction in overbite and maxillary and mandibular incisor proclination during treatment (T1-T3). The center of resistance of the maxillary incisor and the incisal edge was significantly intruded in the intrusion arch group during overbite reduction (T1-T2). However, most of the intrusion of the center of resistance was lost by the end of treatment (T2-T3). Both treatment groups experienced a reduction in maxillary incisor display and flattening of the smile arc during overbite reduction. CONCLUSIONS: Both overbite reduction methods caused a decrease in incisor display and flattening of the smile arc. Smiles were improved in some patients by the end of treatment. However, reduction in incisor display persisted. Clinicians should take precautions to prevent negative effects of overbite reduction.

Laparoscopic Diamond Antroduodenostomy for Postcorrosive Pyloric Cicatrization: A Novel Approach.

BACKGROUND: Traditionally, Billroth I procedure or bypass gastrojejunostomy were the recommended approaches for management of postcorrosive complete gastric outlet obstruction (GOO), whereas Heineke Mickulicz pyloroplasty was recommended for moderate mucosal injury with partial cicatrization. In this study, laparoscopic diamond antroduodenostomy was carried out as an alternative minimally invasive approach for cases with pyloric cicatricial obstruction. PATIENTS AND METHODS: Between January and December 2017, children who were referred to Pediatric Surgery Department, Cairo University Hospital, with GOO as a consequence of caustic liquid ingestions were included in this study. Laparoscopic diamond antroduodenostomy was performed for the presented cases. RESULTS: Through the year 2017, 5 cases were approached with laparoscopic diamond antroduodenostomy. Isolated pyloric cicatrization was evident in 4 cases, whereas synchronous insult to thoracic esophagus and pylorus was manifest in the fifth case. Laparoscopic feeding jejunostomy completed the procedure for the case with esophageal stricture. Contrast study-24 hour postoperatively-assured no radiological leaks in the presented cases, where enteral feeding was gradually commenced, and patients discharged home a day later. After a mean follow-up of 13.5 months, neither recurrence of obstructive symptoms nor dumping was displayed. Cosmetic outlook inherent to the minimally invasive approach was appreciated by the parents. CONCLUSION: Laparoscopic diamond antroduodenostomy is a feasible approach for management of postcorrosive pyloric obstruction. It allowed early enteral feeding, with no dumping symptoms, in addition to the fundamental advantages of minimally invasive surgery. A bigger series and longer follow-up is recommended to verify the reported results.

Incidence and Risk Factors of Rachitic Genu Varus in Preschool Children in a Paediatric Health Institute in Egypt as One of the Developing Countries.

BACKGROUND: Rachitic genu varus is one of the common conditions among the Egyptian children, despite the shinning sun of Egypt all the year. PURPOSE: The aim of the study was to estimate the incidence of rachitic genu varus among the Egyptian children, and to assess the risk factors contributing to it. PATIENTS AND METHODS: This prospective study recruited a total of 250 consecutive children, being 130 males and 120 females, with rachitic bow legs or genu varus, between 2 - 4 years of age, together with 250 controls of the same age group, out of a total number of 1900 children with other types of rickets, and other children's orthopaedic diseases, who presented to the National Institute of Neuromotor System in Egypt between September 2014 to September 2015. The cases and the controls were subjected to clinical, laboratory biochemical, and plain radiographic examinations. Their parents were subjected to epidemiological, maternal, and nutritional questionnaires. RESULTS: The calculations revealed high incidence (13.1%) of rachitic genu varus. The risk factors were low socioeconomic status, insufficient family income, poor housing conditions, lack of exposure to sunlight due to cultural practices, sole breast feeding, and inadequate supplementation of vitamin D to the children and the pregnant women. The p value was <0.05. CONCLUSION: Vitamin D deficiency rachitic genu varus is a multifactorial condition in Egypt. Raising the standard of living, level of education, housings, and dietary supplementation of vitamin D to the pregnant women and infants are the solution.

Outcomes of liver transplantation in patients with hepatorenal syndrome.

Hepatorenal syndrome (HRS) plays an important role in patients with liver cirrhosis on the wait list for liver transplantation (LT). The 1 and 5-year probability of developing HRS in cirrhotic with ascites is 20% and 40%, respectively. In this article, we reviewed current concepts in HRS pathophysiology, guidelines for HRS diagnosis, effective treatment options presently available, and controversies surrounding liver alone vs simultaneous liver kidney transplant (SLKT) in transplant candidates. Many treatment options including albumin, vasoconstrictors, renal replacement therapy, and eventual LT have remained a mainstay in the treatment of HRS. Unfortunately, even after aggressive measures such as terlipressin use, the rate of recovery is less than 50% of patients. Moreover, current SLKT guidelines include: (1) estimation of glomerular filtration rate of 30 mL/min or less for 4-8 wk; (2) proteinuria > 2 g/d; or (3) biopsy proven interstitial fibrosis or glomerulosclerosis. Even with these updated criteria there is a lack of consistency regarding long-term benefits for SLKT vs LT alone. Finally, in regards to kidney dysfunction in the post-transplant setting, an estimation of glomerular filtration rate < 60 mL/min per 1.73 m(2) may be associated with an increased risk of patients having long-term end stage renal disease. HRS is common in patients with cirrhosis and those on liver transplant waitlist. Prompt identification and therapy initiation in transplant candidates with HRS may improve post-transplantation outcomes. Future studies identifying optimal vasoconstrictor regimens, alternative therapies, and factors predictive of response to therapy are needed. The appropriate use of SLKT in patients with HRS remains controversial and requires further evidence by the transplant community.

Minimally invasive single-session double-level rotational osteotomy of the forearm bones to correct fixed pronation deformity in congenital proximal radioulnar synostosis.

BACKGROUND: Congenital proximal radioulnar synostosis is the most common congenital disease of the elbow joints and forearms. METHODS: This was a prospective study of 12 consecutive children (14 forearms) who presented to the National Institute of Neuromotor System in Egypt between September 2012 and September 2013 with severe congenital proximal radioulnar synostosis, having a mean pronation deformity of 70.7° (range 60°-85°), and who underwent operative correction by single-session double-level rotational osteotomy and percutaneous intramedullary K-wires of both the radius and ulna. Ten forearms were type III, and four were type II according to Cleary and Omer classification. The mean age at the time of surgery was 5 years and 2 months (range 4 years and 10 months to 6 years and 5 months). They were evaluated for functional results after rotational corrective osteotomy at a mean interval of 30.4 months (range 24-36 months) by physical examination and radiographs. RESULTS: All children had a mean pronation deformity correction of 59.8° (range 30°-90°) reaching a final position of 20°-30° of pronation in the affected dominant extremities and 20° of supination in the affected non-dominant extremities after osteotomy. All children showed improvement in functional activities, with no loss of correction or non-union in any child, and no circulatory disturbances, neuropathies, or hypertrophic scars. CONCLUSION: Minimally invasive single-session double-level rotation osteotomy of the proximal ulna and distal radius with percutaneous intramedullary K-wire fixation is a safe, technically simple and efficient procedure which corrects pronation deformity.

An approach to outreach patients with von Willebrand disease in Egypt by targeting women with heavy menstrual bleeding and/or bleeding symptoms.

von Willebrand disease (VWD) is frequently ignored as a cause of menorrhagia. We investigated Egyptian women complaining of heavy menstrual bleeding (HMB) and/or other bleeding symptoms to detect potential VWD cases. Seventy-five female patients complaining of HMB and/or bleeding symptoms and 38 age-matched healthy female controls went through a family history questionnaire, a physical examination and were evaluated for bleeding score, pictorial blood assessment chart (PBAC), complete blood count, serum ferritin, blood group, prothrombin time, activated partial thromboplastin time, factor VIII (FVIII) activity, von Willebrand factor (VWF) ristocetin cofactor (RCo) activity, antigen (Ag), and RCo/Ag ratio. Sixty-eight of 75 patients presented with HMB, out of which 46 had no organic pathology and 7 presented other bleeding symptoms. Six patients were diagnosed with VWD, three with HMB, two with other bleeding symptoms and one with family history of VWD. Two related VWD patients were diagnosed in the control group. There were significant differences in bleeding and PBAC scores, ferritin level, FVIII activity, VWF:RCo and VWF:Ag between VWD patients and controls. This study indicated a high prevalence of VWD among patients with HMB without organic pathology (6.5%) and demonstrated the sensitivity of diagnostic parameters of VWD patients in an outreach campaign. The inexpensive bleeding and PBAC scoring systems are valuable to exclude cases without objective bleeding symptoms. Raising gynaecologists awareness about hereditary bleeding disorders is important to ensure a proper diagnosis and possible referral of these patients. Management of these patients with comprehensive medical care services under a multidisciplinary team would be ideal.

Simultaneous femoral and tibial lengthening in combined congenital complete fibular hemimelia and congenital short femur using Ilizarov ring external fixator.

PURPOSE: The combination of fibular hemimelia with congenital short femur worsens the limb length discrepancy which requires extensive femoral and tibial lengthening. PATIENTS AND METHODS: Eight patients having unilateral lower extremity shortening presented to the National Institute of Neuromotor System, Egypt, between September 2008 and September 2010 and underwent single session femoral and tibial lengthening using Ilizarov ring external fixator technique. Consolidation of the femoral and tibial required length gain was evident in the radiographic follow-up. CONCLUSION: Extensive limb length discrepancy can be managed by simultaneous femoral and tibial lengthening. LEVEL OF EVIDENCE: The study is type IV clinical evidence.

Single-event multilevel acute total correction of complex equinocavovarus deformity in skeletally mature patients with spastic cerebral palsy hemiparesis.

Complex multiplanar ankle/foot deformity as equinocavovarus is a common problem in patients with spastic cerebral palsy hemiparesis. The data from 30 consecutive patients (30 feet), treated between March 2009 and March 2010, with equinocavovarus and toe clawing secondary to spastic cerebral palsy hemiparesis, aged 16 to 18 years, were analyzed clinically and radiographically. All the patients had received conservative physiotherapy treatment and ankle/foot orthoses before undergoing combined soft tissue and bony surgical procedures performed in a single session to correct the complex toe clawing, cavus, varus, and equinus deformities. Preoperative measurements of certain foot angles were compared with their corresponding postoperative values. A grading system for evaluation of the results using a point scoring system was used to accurately evaluate both the clinical and the radiographic results after an average follow-up period of 2.5 years. Of the 30 patients (30 feet), 18 (60%) had excellent, 9 (30%) good, 3 (10%) fair, and 0 had poor outcomes. Neither vascular problems nor nonunion occurred. Significant improvement was seen postoperatively (p < .0333). Neither staged surgical procedures nor gradual distraction techniques using external fixators are ideal modalities to correct complex ankle/foot equinocavovarus deformity in patients with spastic cerebral palsy. Single-event, multilevel surgery with complete soft tissue and bony correction appears to be the treatment of choice in such cases. It shortens the treatment period and avoids patient dissatisfaction associated with multiple procedures, without major complications.

Outcome and relapse risks of thrombotic thrombocytopaenic purpura: an Egyptian experience.

BACKGROUND: Thrombotic thrombocytopaenic purpura (TTP) is a rare life-threatening disease. Plasma exchange has significantly decreased the mortality from this disease, which still tends to recur in a substantial proportion of patients. This study describes the clinical spectrum and response to treatment and explores the risks of relapse in a cohort of patients. METHODS: Patients treated for TTP at the Clinical Haematology Unit, Cairo University, Egypt, between 2000 and 2008 were identified. Complete demographic, clinical history and full clinical examination, laboratory, treatment modalities and duration, and outcome data were collected and analysed. The follow-up duration was 24 months. RESULTS: 30 patients; 13 men (43%) and 17 women (57%) with a median age of 42 years were treated for 46 episodes of TTP. The median duration of disease onset to diagnosis for the first episode was 7 days. Twenty-three patients (76.66%) were diagnosed as idiopathic primary and seven patients (23.33%) were secondary TTP. Four patients died during the first 24 h. Of the 26 patients, 22 (85.6%) achieved remission with an average of 7.55 plasma exchange sessions, Another nine patients had 25 relapses (mean 2.7). Splenectomy was performed in three patients (11.5%). The 24-month overall survival was 80%. The initial low platelet count and high LDH were the only two statistically significant relapse predictors. CONCLUSIONS: The current results conform to the reported literature on the outcome of TTP. The very early mortality due to late referral highlights the need of education about the disease among primary healthcare providers.