Pentalogía de Cantrell diagnosticada en el primer trimestre: reporte de caso / Cantrell's pentalogy diagnosed in the first trimester: case report

La pentalogía de Cantrell es una rara anomalía congénita caracterizada por la asociación de ectopia cordis con defectos en la pared toracoabdominal, el diafragma, el esternón y pericárdicos, y anomalías cardíacas intrínsecas. En diagnóstico prenatal, la ecografía se utiliza sistemáticamente entre las 11 y 14 semanas de gestación, evaluando marcadores de alteraciones cromosómicas como la sonolucencia nucal, el hueso nasal y la morfología patológica del ductus venoso, entre otros. Además, permite examinar la anatomía fetal y diagnosticar anomalías mayores, como acrania-anencefalia, holoprosencefalia, defectos de la pared abdominal y toracoabdominal, entre los que se incluye la pentalogía de Cantrell. Se reporta un feto con los hallazgos clásicos de pentalogía de Cantrell, que fue expulsado a las 13 semanas de gestación bajo protocolo de interrupción voluntaria del embarazo. Madre de 23 años, G1P0, sin exposiciones teratogénicas, en cuyo feto se encontró ectopia cordis, asas intestinales e hígado por fuera de la cavidad abdominal en las 10 y 12 semanas de gestación. El objetivo de este estudio es aportar a la literatura un reporte de pentalogía de Cantrell, siendo el primero reportado en Colombia en el primer trimestre de gestación, mostrando la importancia de la ecografía sistemática durante este periodo, en el marco de la posibilidad de interrupción voluntaria del embarazo.

Cantrells pentalogy is a rare congenital anomaly characterized by the association of ectopia cordis with intrinsic cardiac anomalies and various anatomical defects found in the thoracoabdominal wall, diaphragm, sternum and pericardium. Ultrasound is used routinely between 11 and 14 weeks of gestation during prenatal diagnosis. It evaluates markers of chromosomal alterations such as nuchal sonolucency, the nasal bone, and the pathological morphology of the ductus venosus, among others. Furthermore, it allows the diagnosis of altered fetal anatomy and major abnormalities such as acrania-anencephaly, holoprosencephaly, abdominal and thoraco-abdominal wall defects including Cantrells pentalogy. In this case report, we present a fetus with the classic findings of Cantrells pentalogy, which was expelled during the 13th week of gestation under the protocol of voluntary interruption of pregnancy. The mother, a 23-year-old woman, G1P0, without teratogenic exposures, in whom during the routine ultrasound of the 10th and 12th weeks of gestation ectopia cordis, intestinal loops and liver outside the abdominal cavity were found on the fetus. The main objective of this study is to contribute to the literature a case report of pentalogy of Cantrell, diagnosed through prenatal ultrasound, being the first reported in Colombia during first trimester of gestation, showing the importance of routine ultrasound, in the context of access to a voluntary termination of pregnancy.

Outcomes of lensectomy with glued intraocular lens and factors associated with failure in eyes with spherophakia and glaucoma

Purpose: To evaluate the outcomes of lensectomy with a glued intraocular lens (IOL) in spherophakic eyes with secondary glaucoma and assess factors associated with failure. Methods: We prospectively evaluated outcomes of lensectomy with glued IOL in 19 eyes with spherophakia and secondary glaucoma (intraocular pressure (IOP) ?22 mm Hg and/or glaucomatous optic disc damage) between 2016 and 2018. The vision, refractive error, IOP, antiglaucoma medications (AGMs), optic disc changes, need for glaucoma surgery, and complications were assessed. Success was defined as complete when IOP was ?5 and ?21 mmHg without AGMs; qualified success as similar IOP with up to 3 AGM; the need for >3AGM/additional surgery for IOP control was considered a failure. Results: Preoperatively, the median (interquartile range: IQR) age was 18 (13.5–30) years. IOP was 16 (14–22.5) mmHg on a median of 3 (2,3) AGMs. Median postoperative follow up was 27.7 months (11.9, 39.7). Postsurgery, most patients achieved emmetropia, with significantly decreased refractive error from a median spherical equivalent of ?12.5D to + 0.5D, P < 0.0002. The complete success probability was 47% (95% confidence intervals (CIs): 29–76%) at 3 months and was 21% (8 ? 50%) at 1 year and 3 years. The qualified success probability was 93% (82–100%) at 1 year, which reduced to 79% (60–100%) in 3 years. None of the eyes had any retinal complications. The higher number of preoperative AGM was found to be a significant risk factor (p < 0.02) for the failure of complete success. Conclusion: One?third of the eyes had IOP control without the need for AGM postlensectomy with glued IOL. Surgery resulted in significant improvement in visual acuity. The higher number of preoperative AGM was associated with poor glaucoma control after glued IOL surgery

Kissing microvitreoretinal blade technique: A novel approach for safe and effective endocapsular lens aspiration in microspherophakia

We herein describe the kissing MVR technique in cases of microspherophakia for safe and effective endocapsular lens aspiration. Microspherophakia is associated with abnormally lax and broken zonules, which pose a great challenge to the operating surgeon, especially while creating the openings in the capsular bag. In this novel technique, simultaneous use of two 23 G MVR blades reduces the above problem associated with the severely mobile lens. Here, one MVR blade stabilizes the capsular bag and, at the same time, provides counter-traction for the opposite MVR blade while puncturing the capsular bag. Furthermore, the creation of openings in the lens at the equator or just posterior to it is beneficial as the capsule is relatively thicker and stronger at this location. This also minimizes the risk of premature extension to the anterior or posterior capsule, thereby avoiding complications like dropped lens matter, vitreous prolapse, and vitreous traction.

Outcomes of surgical intervention in cases of ectopia lentis

Purpose: To evaluate the outcomes of surgical intervention in cases of ectopia lentis. Methods: This retrospective study included all cases of ectopia lentis that presented between June 2015 and March 2019 in a tertiary care center. They were reviewed retrospectively. The corrected distance visual acuity (CDVA), severity of lens subluxation, type of surgery, intra?operative and post?operative complication, and specular count were recorded. Results: Seventy?eight eyes of 57 cases with a mean age at surgery of 14.73 years were analyzed. Intra?lenticular lens aspiration was the most common (n?62/78; 79.5%) surgical procedure followed by lens aspiration, intra?capsular cataract extraction, phaco?aspiration, and pars?plana lensectomy. Simultaneous intra?ocular lens (IOL) implantation was performed in 46.2% (n?32/78) of the eyes. The mean CDVA improved from 0.85 ± 0.55 logMAR to 0.44 ± 0.29 logMAR at 6 weeks follow?up. The post?operative CDVA was significantly better in the pseudo?phakic group compared to the aphakic group (p?0.02). The patient’s age at the time of surgery and the degree of subluxation did not impact the final visual outcome. Intra?operative complication included vitreous hemorrhage (n?1) and lens matter drop (n?1). Post?operative complications were noted in 26.9% of the eyes (n?21/78) with a higher complication rate in the pseudo?phakic group (p?0.00). A second intervention was required in 7.7% of the eyes (n?6/78). Conclusion: Age and degree of subluxation at the time of surgery do not influence the final visual outcome in cases of ectopia lentis undergoing lens extraction surgery. IOL implantation results in better visual outcomes but is associated with a high complication rate.

Retropupillary iris claw lens versus Gore-Tex assisted scleral fixated intraocular lens in children with large lens subluxations

Purpose: To compare the functional outcome of retropupillary iris claw lenses (RPIC?IOL) and scleral fixated intraocular lenses (SFIOL) in children with large lens subluxations. Methods: Sixty eyes of patients between 6 and 18 years of age having ?7 clock hour lens subluxation were included and equally divided into group A (RPIC?IOL implantation) and group B (Gore?Tex sutured SFIOL implantation). Cases with anterior and posterior segment abnormalities, trauma and glaucoma were excluded. Primary outcome was improvement in best?corrected visual acuity (BCVA) at 1.5 years. Secondary outcomes were assessment of intraocular lens (IOL) tilt, mean change in astigmatism at 1.5 years, and median operating time. All surgeries were performed by the same surgeon. Results: The mean improvement in BCVA in group A was 0.28 ± 0.41 logMAR and group B was 0.44 ± 0.45 logMAR (P = 0.3). Significant IOL tilt was seen in 4 eyes in group A (13.33%) and 5 eyes in group B (16.66%) (P = 0.120). Mean change in astigmatism was 4.38 ± 5.9D in group A and 4.91 ± 4.4D in group B (P = 0.299). The median operating time was 40 min in group A and 90 min in group B (P < 0.001). No significant posterior segment complications were seen in either technique. Conclusion: Both procedures had comparable visual outcomes. RPIC?IOL implantation was relatively quick and comparatively easier; it may be preferred in cases with high risk of retinal detachment.

Ectopia renal cruzada fusionada. Reporte de caso

La ectopia renal cruzada fusionada es una entidad rara dentro del espectro de anomalías congénitas del desarrollo del tracto urinario, con una incidencia estimada de aproximadamente uno de cada 1000 nacidos vivos y una predilección masculina en una proporción de dos a uno al comparar con la población femenina. Esta entidad suele ser asintomática en la mayoría de los pacientes. La presencia de síntomas suele estar ligada a un espectro de complicaciones, dentro de los cuales cabe destacar la hidronefrosis, infecciones, reflujo besico ureteral y nefrolitiasis. Reconociendo la baja incidencia de esta anomalía y la importancia de su identificación, en el presente artículo reportamos un caso de nuestra institución, observado como hallazgo incidental en una tomografía abdominopélvica contrastada por dolor abdominal inespecífico. (provisto por Infomedic International)

Fused renal crossover ectopia is a rare entity within the spectrum of congenital anomalies of urinary tract development, with an estimated incidence of approximately one in 1000 live births and a male predilection in a two to one ratio when compared to the female population. This entity is usually asymptomatic in most patients. The presence of symptoms is usually linked to a spectrum of complications, including hydronephrosis, infections, bile-ureteral reflux and nephrolithiasis. Recognizing the low incidence of this anomaly and the importance of its identification, in this article we report a case from our institution, observed as an incidental finding in a contrasted abdominopelvic tomography for non-specific abdominal pain. (provided by Infomedic International)

Ectopia cordis abdominal. Apropósito de un caso

Resumen Introducción: La ectopia cordis es una malformación congénita poco frecuente y grave caracterizada por el desplazamiento del corazón fuera de la cavidad torácica, Existe discreto predominio en el sexo femenino y se asocia a teratógenos no potentes y relacionado también a factores genéticos, su diagnóstico prenatal es muy importante y su pronostico es desfavorable en casi la totalidad de los casos. Objetivo: Describir el manejo con una paciente con ectopia cordis en Letsholathebe Memorial Hospital II Republica de Botswana. Descripción: Se describe el manejo por parte del pediatra y el cardiólogo desde su diagnóstico desde su nacimiento hasta sus ingresos hospitalarios. Conclusiones: Ectopia cordis es una malformación congénita rara que tiene un mal pronóstico incluso después del enfoque quirúrgico y su gravedad está relacionada con la presencia de otras anomalías.

ABSTRACT Introduction: Ectopia cordis is a rare and severe congenital malformation characterized by the displacement of the heart outside the chest cavity, there is discreet predominance in the female sex and is associated with non-potent teratogens and also related to genetic factors, its prenatal diagnosis is very important and its prognostic is unfavorable in almost all cases. Objective Describe management with a patient with ectopia cordis at Letsholathebe Memorial Hospital II Republic of Botswana. Description: It describes the management by the pediatrician and the cardiologist from his diagnosis from birth to his hospital admissions. Conclusions: Ectopia Cordis is a rare congenital malformation that has a poor prognosis even after the surgical approach and its severity is related to the presence of other abnormalities.

Clinical profile and outcome of ocular manifestation in Marfans syndrome in India

Purpose: Marfan syndrome (MFS) is a genetic disorder associated with considerable morbidity and mortality. Presently, well?documented information on this condition is not available in India. Methods: In this retrospective cohort study, we recruited patients with clinically diagnosed MFS who presented to the outpatient department using revised Ghent nosology. We retrieved complete ophthalmic information, including vision, anterior and posterior segments, exported from electronic medical records, and relevant investigations, surgical details, and follow?up data were obtained in a specific, pretested format. Results: Our cohort consisted of 86 eyes of 43 patients and had a male preponderance. The prevalence was 20.5 per 100,000 individuals. The mean age of the patients was 23.9 years. All eyes were treated either optically with refraction or surgically using lensectomy and vitrectomy with suture supported scleral fixated intraocular lens (IOL), which significantly affected the visual outcome (P = 0.000). Conclusion: Although considered a rare disease, MFS is commonly found in the ophthalmological setting. Refraction and surgery (lensectomy with scleral fixated IOL) may improve the vision significantly

Tiroides Ectópica Lingual: caso Clínico / Lingual Ectopic Thyroid: a clinical case

La tiroides ectópica lingual es una patología muy poco frecuente, producida por la detención en el descenso normal de la glándula durante el desarrollo embrio-nario. La localización lingual de tejido tiroideo es la más común entre las tiroides ectópicas o aberrantes. Esta enfermedad puede ser asintomática pero, cuan-do los signos y síntomas están presentes, guardan estrecha correlación con la localización de la lesión y son proporcionales a su tamaño. El diagnóstico debe realizarse clínicamente y con el complemento de es-tudios por imágenes y endocrinológicos. En los aná-lisis de laboratorio se debe incluir dosaje de las hor-monas TSH, T4 libre y T3, vinculadas con la función tiroidea. Las biopsias deben evitarse ya que causan desequilibrio en la producción hormonal de la glándu-la y peligro de profusas hemorragias. En este artículo se desarrolla una descripción de las generalidades de la tiroides ectópica lingual, y se presenta un caso clínico de un niño con un tumor lingual, que fue deri-vado por su médica pediatra a cirugía para realizar una biopsia. Asimismo, se comenta la importancia que tiene para el odontólogo conocer esta patología a fin de poder evitar sus posibles complicaciones (AU)

Lingual thyroid is a rare disorder produced by a failure in the descent of thyroid gland to its normal position during embryological development. Lingual localization of thyroid tissue is the most common among the ectopic or aberrant thyroids. This condition can be asymptomatic, although when symptoms take place, they are connected to the lesion location and depend on its size. Diagnosis should be made clinically and complemented with imaging and endocrine studies. Laboratory analysis must include dosage of TSH, free T4 and T3, thyroid function-linked hormones. Due to the possible imbalance in the gland hormone production and the risk of massive bleeding, biopsy should be avoided. In this article, a brief description of lingual ectopic thyroid generalities is developed and a clinical case of a 7-years old child is provided. Additionally, dentistry importance of knowing this condition is commented, in order to prevent its possible complications (AU)

Traboulsi syndrome caused by a newly discovered homozygous mutation of the ASPH gene: a case report / 中华实用儿科临床杂志

Clinical data of a child with Traboulsi syndrome diagnosed in Affiliated Haikou Hospital of Xiangya Medical College, Central South University in November 2019 were retrospectively analyzed.A 9-year-old female patient presented with vision loss for 3 years.Ectopic lens and a special facial appearance were the main manifestations.Genetic testing showed a homozygous mutation at the c. 1126C > T site of the ASPH gene in the present case, and finally, Traboulsi syndrome was diagnosed.The clinical manifestation of Traboulsi syndrome is not typical.Mastering the main diagnostic points is helpful to improve the efficacy of clinical diagnosis and treatment.c.1126C>T mutation of the ASPH gene has not been reported in China and abroad.It is a newly discovered mutation that enriches the ASPH gene spectrum.

New clues to understand gastroschisis. Embryology, pathogenesis and epidemiology / Nuevas pistas para comprender la gastrosquisis. Embriología, patogénesis y epidemiología

Abstract gastroschisis is a congenital structural defect of the abdominal wall, most often to the right of the umbilicus, through which the abdominal viscera protrude. Its developmental, etiological and epidemiological aspects have been a hot topic of controversy for a long time. However, recent findings suggest the involving of genetic and chromosomal alterations and the existence of a stress-inducing pathogenetic pathway, in which risk factors such as demographic and environmental ones can converge. . We have conducted a review of the medical literature that gathers information on the embryonic development of the ventral body wall, the primitive intestine, and the ring-umbilical cord complex, as well as on the theories about its origin, pathogenesis and recent epidemiological evidence, for which we consulted bibliographic databases and standard search engines

Resumen La gastrosquisis es un defecto estructural congénito de la pared abdominal, localizado con mayor frecuencia a la derecha del ombligo, a través del cual sobresalen las vísceras abdominales. Durante mucho tiempo, sus aspectos evolutivos, etiológicos y epidemiológicos han sido un tema candente de controversia, aunque hallazgos recientes sugieren la participación de alteraciones genéticas, cromosómicas, y la existencia de una vía patogénica inductora de estrés, en la que factores de riesgo como los demográficos y ambientales pueden converger. Con el objetivo de ampliar la frontera del conocimiento sobre una malformación que ha mostrado una creciente prevalencia global, hemos efectuado una revisión que incluye información, del desarrollo embrionario de la pared corporal ventral, el intestino primitivo, el complejo anillo-cordón umbilical, y de las teorías acerca de su origen, patogénesis e información epidemiológica reciente.

Torsión esplénica con localización inusual, reporte de caso / Splenic torsion with unusual location, case report

Resumen El bazo errante es una entidad clínica muy poco frecuente en niños, representando menos del 0.25% de todas las esplenectomías. Puede ocurrir por la ausencia congénita de ligamentos suspensorios del bazo, o debido a una laxitud adquirida de los mismos. El espectro clínico va desde condición asintomática con hallazgo incidental, hasta presentación de abdomen agudo. El conocimiento de esta condición y de sus hallazgos radiológicos, juegan un papel esencial para lograr un diagnóstico correcto y tratamiento quirúrgico oportuno. La preservación del bazo más esplenopexia es el procedimiento de elección. Se presenta un paciente de 4 años con antecedente de cirugía antirreflujo, en quien se encontró bazo ectópico errante con torsión esplénica, que fue manejado exitosamente con conservación del bazo y esplenopexia. MÉD.UIS.2021;34(1): 113-18

Abstract Wandering spleen is an uncommon clinical entity in children, representing less than 0,25% of all splenectomies. It is caused by laxity or absence of the supporting splenic ligaments. Its clinical spectrum varies from asymptomatic disease with incidental finding to acute abdominal pain due to torsion. The knowledge of this condition and its radiological findings plays a crucial role in reaching the correct diagnosis and timely surgical treatment. Spleen preservation with splenopexy is recognized as the procedure of choice. We report a case of a four year old patient with a history of anti-reflux surgery, with a wandering spleen and splenic torsion, successfully managed with spleen conservation and splenopexy. MÉD.UIS.2021;34(1): 113-18

A propósito de un caso: Resolución quirúrgica en dos tiempos de Pentalogía de Cantrell / Case Report: Two-stage surgical resolution of Cantrell's Pentalogy

INTRODUCCIÓN: La pentalogía de Cantrell abarca la presencia de cinco defectos congénitos que representan un gran desafío para los cirujanos. Las anomalías del corazón, pericardio, diafragma, esternón y pared abdominal anterior son los hallazgos principales. Su incidencia es baja, sin embargo, es fundamental identificarla oportunamente para adoptar una terapia adecuada para todos los defectos descritos, puesto que se reporta una mortalidad elevada. CASO CLÍNICO: Se trató de un recién nacido a término, de sexo masculino, con antecedentes de defecto de la pared abdominal compatible con onfalocele detectado mediante ecografía prenatal. Tras cesárea programada, se evidenció defecto en la pared abdominal a través del cual emergían el lóbulo hepático izquierdo, intestinos y corazón; el esternón además presentaba una fisura baja. En ecocardiograma se evidenció foramen oval permeable, insuficiencia tricuspídea leve e hipertensión pulmonar severa. EVOLUCIÓN: Se decidió su manejo quirúrgico inmediato. Se colocó silo, con cierre progresivo de la línea media en 7 días. En segundo tiempo quirúrgico, se corrigió el defecto diafragmático y pericárdico con prótesis de pericardio bovino. Pese a la evolución adecuada de la cirugía, a los 28 días, el paciente presentó cianosis súbita, sin responder a maniobras de reanimación y falleció. CONCLUSIÓN: La Pentalogía de Cantrell es una enfermedad rara, con características clínicas, anatómicas y embriológicas peculiares, representa un desafío único para los cirujanos. Su diagnóstico temprano, así como el seguimiento durante el embarazo, la planificación de una cesárea en un centro de alto nivel y la aproximación quirúrgica inmediata con un equipo multidisciplinario, son componentes clave en el manejo integral de pacientes con Pentalogía de Cantrell.

BACKGROUND: Cantrell's pentalogy includes the presence of five birth defects that represent a great challenge for surgeons. Abnormalities of the heart, pericardium, diaphragm, sternumand anterior abdominal wall are the main findings. Its incidence is low, however, it is essential to identify Cantrell´s pentalogy timely to adopt an adequate therapy for all specific defects, since it has high mortality. CASE REPORT: The patient was a full- term male newborn, with a history of abdominal wall defect compatible with an omphalocele detected by prenatal ultrasound. After the caesarean section, the abdominal wall defect was notable, the left liver lobe, intestines and heart emerged through it, the sternum also had a low fissure. The echocardiogram revealed a permeable oval foramen, mild tricuspid regurgitation, and severe pulmonary hypertension. EVOLUTION: Immediate surgical management was decided. Silo was placed, with progressive closure of the midline in 7 days. During the second surgical procedure, the diaphragmatic and pericardial defect was corrected with a bovine pericardial prosthesis. Despite the adequate evolution after surgery, at day 28 he presented with sudden cyanosis and didn't respond to cardiopulmonary resuscitation and died. CONCLUSIONS: Cantrell's Pentalogy is a rare disease, with peculiar clinical, anatomical and embryological characteristics, it represents a unique challenge for surgeons. Early diagnosis, as well as follow-up during pregnancy, planning a cesarean section in a high-level center and immediate surgical approach with a multidisciplinary team, are the key components in the management of patients with Cantrell's Pentalogy.

Forgotten double J stent in crossed renal fused ectopia: a rare case report

Ureteral double J (DJ) sent has now become one of the most commonly used tools in endourology. Complications are bound to occur if forgotten to remove, such as encrustations, infection, migration, renal dysfunction, hydronephrosis. Crossed renal fused ectopia is a very rare congenital malformation due to abnormal kidney ascent with fusion during embryogenesis in the first trimester. we report a very rare case of forgotten DJ stent in crossed fused ectopia, in 66 years old diabetic patient post left ureterorenoscopy (URS) done 3 years back. Retrograde intrarenal surgery (RIRS) was done for DJ stent removal right.

Diagnóstico prenatal de Pentalogía de Cantrell: reporte de caso / Prenatal diagnosis of Pentalogy of Cantrell: A case report / Prenatal diagnosis of Pentalogy of Cantrell: A case report

RESUMEN La pentalogía de Cantrell es la conjunción de cinco defectos congénitos (defectos del corazón, pericardio, diafragma, esternón y la pared abdominal anterior), que representa un desafío único para los gineco-obstetras. Aunque la incidencia es pequeña, es fundamental identificarlo en el primer trimestre. En este reporte se comunica un caso de pentalogía de Cantrell diagnosticado en su primer control prenatal en el segundo trimestre de gestación.

ABSTRACT The Pentalogy of Cantrell is the conjunction of five congenital defects (defects of the heart, pericardium, diaphragm, sternum and anterior abdominal wall) which represents a unique challenge for the obstetrician gynecologist. Although its incidence is small, it is essential to identify it early in the first trimester. A case of Pentalogy of Cantrell is reported, diagnosed in the first prenatal control in the second trimester of pregnancy.

Management of Chiari I Deformity in Children and Adolescents: A Report from the Consensus Taskforce of the Brazilian Society of Pediatric Neurosurgery

Much controversy remains on the current management of Chiari I deformity (CID) in children, with many clinical, surgical and ethic-legal implications. The Brazilian Society of Pediatric Neurosurgery (SBNPed, in the Portuguese acronym) has put together a panel of experts to analyze updated published data on the medical literature about this matter and come up with several recommendations for pediatric neurosurgeons and allied health professionals when dealing with CID. Their conclusions are reported herein, along with the respective scientific background.

Peripheral iridectomy for preventing iris-related complications in glued intraocular lens surgery in children

Purpose: To assess the role of surgical peripheral iridectomy (PI) in preventing iris-related complications associated with glued intraocular lens (GIOL) surgery in children with bilateral ectopia lentis. Methods: Nonrandomized interventional case series of 34 eyes of 17 children (<15 years of age) who underwent pars plana lensectomy (PPL) and GIOL surgery between January 2013 and December 2016. Eyes with surgical PI (January 2013–June 2015) were compared with those without surgical PI (July 2015–December 2016). The primary outcome measure of the role of surgical PI in GIOL surgery was to account for complications such as optic capture, secondary glaucoma, intraocular lens (IOL) dislocation, or repeat surgery. The secondary outcomes were changes in the best-corrected visual acuity (BCVA). Results: The mean age at surgery was 8.8 years (range: 3.5–15 years). Surgical PI was conducted in 15 eyes. Among the 19 eyes without PI, 9 eyes had complications (optic capture –6; rise in IOP –4; IOL subluxation –4; repeat surgery –5). The complications were significantly less in the PI group, P = 0.02. There was a statistically significant improvement in BCVA (P = 0.0001) in all the patients. The mean presenting BCVA was 0.99 (±0.79) logMAR (Snellen ? 20/200) and post BCVA was 0.40 (±0.50) (Snellen ? 20/50). The mean preoperative refraction was ? 9 D (±8D) (range: ?5 D to ?23D) and postoperative was ?1 (±1.15) D. The mean follow-up was 25.4 months. Conclusion: Surgical PI along with GIOL surgery in children undergoing PPL is shown to reduce optic-capture-related complications.

Bimanual irrigation-aspiration for ectopia lentis and use of a small incision for 4-point scleral-sutured foldable intraocular lens and anterior vitrectomy in patients with Marfan syndrome

Purpose: This study evaluated bimanual intracapsular irrigation-aspiration for ectopia lentis with use of a small incision for 4-point scleral fixation of a foldable posterior-chamber intraocular lens (IOL) and anterior vitrectomy in patients with Marfan syndrome. Methods: We performed a retrospective study of 18 eyes from 10 patients with Marfan syndrome who underwent surgical intervention for ectopia lentis at our clinic between July 2012 and September 2018. In this study, intraoperative and postoperative complications, uncorrected visual acuity, best-corrected visual acuity, spherical equivalent, intraocular pressure, and endothelial cell density were evaluated. Results: No intraoperative complications were reported. In all cases, early postoperative evaluation revealed a clear cornea, round pupil, and well-centered IOL. Mean logMAR uncorrected visual acuity improved from 1.09 preoperatively to 0.56 postoperatively (P < 0.05). Mean logMAR best-corrected visual acuity improved from 0.45 preoperatively to 0.17 postoperatively (P < 0.05). Aside from transient ocular hypertension, no postoperative complications were reported. Conclusion: The combined surgical technique presented above yields excellent visual outcomes with an extremely low incidence of complications. This approach is simple, safe, and effective in the treatment of ectopia lentis in patients with Marfan syndrome.

Ectopia cordis associated with pentalogy of cantrell-a case report / Ectopia cordis associada à pentalogia de cantrell-relato de caso

Abstract Pentalogy of Cantrell (PC) is a rare congenital anomaly characterized by changes in the mesodermal median structures and congenital heart disease, often with a poor prognosis. In 1958, Cantrell et al2 defined the full spectrum of the syndrome with the following anomalies: defects of the anterior diaphragm, of the lower part of the sternum, of the supraumbilical region and the abdominal wall, of the diaphragmatic pericardium, and various intracardiac congenital abnormalities. The present report describes a case of ectopia cordis associated with PC and the importance of the participation of a multidisciplinary team in the treatment of this condition.

Resumo A pentalogia de Cantrell (PC) é uma rara anomalia congênita caracterizada por alterações nas estruturas medianas mesodérmicas e doenças cardíacas congênitas, cursando muitas vezes com um mau prognóstico. Em 1958, Cantrell et al2 definiram o espectro completo da síndrome com as seguintes anomalias: defeitos do diafragma anterior, da parte inferior do esterno, da região supraumbilical e parede abdominal, do pericárdio diafragmático, e várias anormalidades congênitas intracardíacas. O presente relato relaciona-se a um caso de ectopia cordis associado à PC e à importância da participação de uma equipe multidisciplinar no acompanhamento da doença.