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1.
Pediatr Radiol ; 53(11): 2291-2304, 2023 10.
Artículo en Inglés | MEDLINE | ID: mdl-37466734

RESUMEN

Medical imaging plays a crucial role in the management of conjoined twins. The role of imaging is to explore the anatomy, outline the shared organs and determine whether surgery is feasible. It also serves as a roadmap for successful separation. Additionally, imaging helps with counseling parents about prognosis. This review aims to illustrate recent advances in different imaging modalities and their role in the management of the various types of conjoined twins with an emphasis on relevant tips for optimal imaging.


Asunto(s)
Gemelos Siameses , Embarazo , Femenino , Humanos , Gemelos Siameses/cirugía , Ultrasonografía Prenatal/métodos , Imagen Multimodal , Pronóstico
2.
Eur J Pediatr ; 180(8): 2581-2589, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-34009469

RESUMEN

Congenital haemangioma (CH) is a rare benign vascular tumour presenting at birth with excellent prognosis. Usually, CH regresses without treatment within the first few months of life. Kaposiform Haemangioendothelioma (KHE) is another type of vascular tumours that has been described as benign with locally aggressive potential. Although the diagnosis of vascular tumours is usually straightforward based on typical clinical presentation, yet some confusing similarities may exist with congenital sarcomas.Conclusion: Data of cases managed at the vascular anomaly clinic during the period 2015 through 2019 were retrospectively analysed. The study included three groups of patients: cases diagnosed as congenital haemangioma (9 cases), cases of Kaposiform Haemangioendothelioma who presented in the neonatal period (7 cases), as well as cases of congenital fibrosarcoma (4 cases) that were referred to the vascular anomaly clinic because of apparent similarity with vascular tumours. The hallmark of the study was to compare clinical and imaging features in the three groups to facilitate differentiation and remove diagnostic confusion when managing these rare cases in the future. What is Known: • Congenital haemangioma is a rare benign vascular tumour presenting at birth. • Kaposiform Haemangioendothelioma is another type of vascular tumours that has been described as benign with locally aggressive potential. What is New: • Confusing similarities may exist between vascular tumours and congenital sarcomas.


Asunto(s)
Fibrosarcoma , Hemangioma , Síndrome de Kasabach-Merritt , Diagnóstico Diferencial , Fibrosarcoma/diagnóstico , Hemangioendotelioma , Hemangioma/diagnóstico , Humanos , Recién Nacido , Síndrome de Kasabach-Merritt/diagnóstico , Estudios Retrospectivos , Sarcoma de Kaposi
3.
Neuroradiology ; 62(4): 525-531, 2020 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-31955236

RESUMEN

PURPOSE: To investigate the correlation between the diffusion tensor imaging (DTI) measures and the reading, spelling, writing, rapid naming, memory, and motor abilities in Arabic dyslexic children. This could verify the influence of possible white matter alterations on the abilities of those children. METHODS: Twenty native Arabic-speaking children with dyslexia (15 males and 5 females; 8.2 years ± 1) underwent DTI of the brain on 1.5 T scanner. Diffusion-weighted images were acquired in 32 noncollinear direction. Tractography of the arcuate fasciculus (AF) was performed. Region of interest (ROI)-based approach was also used. Regions encompass superior longitudinal fasciculus (SLF), anterior and superior corona radiata (CR), and posterior limb of internal capsule (PLIC) were analyzed. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were measured. The aptitudes of those children were evaluated by the dyslexia assessment test. These abilities were statistically correlated with the FA and ADC of the AF and other ROIs. RESULTS: The reduction of FA of right AF was related to worse overall reading and related abilities performance. The ADC of right SLF was negatively correlated with memory abilities. The ADC of right PLIC was positively correlated with writing performance. Other relations were also found. CONCLUSION: White matter microstructural DTI measurements in the right AF, right PLIC, SLF, and left anterior and superior CR are correlated to reading, spelling, writing, memory, and rapid naming abilities of the participants. The DTI measures could be promising regarding their use as a biomarker for follow-up in developmental dyslexia.


Asunto(s)
Cognición , Imagen de Difusión Tensora , Dislexia , Sustancia Blanca/diagnóstico por imagen , Anisotropía , Núcleo Arqueado del Hipotálamo/diagnóstico por imagen , Niño , Egipto , Femenino , Humanos , Interpretación de Imagen Asistida por Computador , Masculino , Pruebas Neuropsicológicas
4.
Neuroradiology ; 60(10): 1053-1061, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-30074067

RESUMEN

PURPOSE: Despite its clinical implications, the MRI features of developmental facial paresis (DFP) were described in a few case reports. This study aims to describe MRI features of DFP in relation to the embryological development with a proposed radiological new grading system. METHODS: The clinical records and MRI of the brain and internal auditory canal of 11 children with DFP were retrospectively reviewed. The following sequences were analyzed: axial, oblique sagittal SPACE of the internal auditory canal and brainstem; axial T2, T1WI and coronal T2WI of the brain. The severity of the maldevelopment of the seventh nerve was graded from 0 to 4: 0 = no abnormalities, 1 = unilateral facial nerve hypoplasia, 2 = unilateral facial nerve aplasia, 3 = aplasia or hypoplasia involving facial nerves on both sides, and 4 = facial nerve aplasia or hypoplasia associated with other cranial nerve palsy. RESULTS: Isolated facial nerve palsy was diagnosed in seven patients. It was of grade 1 in five and grade 3 in two. Hypoplasia of the nerve with interrupted course was encountered in two cases. Other associated cranial nerve abnormalities (grade 4) were seen in four patients; two of them were diagnosed previously as Moebius syndrome. In addition to inner ear anomalies, middle and external ear and parotid gland anomalies were described. CONCLUSION: To our knowledge, this is the largest series of patients with DFP that represents a continuum of isolated and combined malformations. Understanding of embryological basis can give insights into the anomalous development of the facial nerve.


Asunto(s)
Enfermedades de los Nervios Craneales/congénito , Enfermedades de los Nervios Craneales/diagnóstico por imagen , Nervios Craneales/anomalías , Parálisis Facial/congénito , Parálisis Facial/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Adolescente , Niño , Preescolar , Femenino , Humanos , Interpretación de Imagen Asistida por Computador , Lactante , Masculino , Estudios Retrospectivos , Índice de Severidad de la Enfermedad
5.
Childs Nerv Syst ; 34(11): 2223-2231, 2018 11.
Artículo en Inglés | MEDLINE | ID: mdl-29850941

RESUMEN

BACKGROUND: Recently, most cases of hydrocephalus are related to obstruction. Accurate localization of the site of obstruction is crucial in determination of the treatment strategy. PURPOSE: To describe the phase-contrast and 3D-DRIVE findings in cases of obstructive hydrocephalus in paediatric patients and to determine their functional and anatomical correlates. MATERIAL AND METHODS: Brain MRIs of 25 patients (2 months to 11 years) with obstructive hydrocephalus were retrospectively reviewed. Phase-contrast and 3D-DRIVE were performed to assess cerebrospinal (CSF) pathways through the aqueduct of Sylvius and subarachnoid spaces. In addition to flow velocity measurement at the aqueduct of Sylvius, functional and anatomical correlation was analysed at the level of aqueduct of Sylvius, infracerebellar CSF space and at the third ventriculostomy using Spearman's rank test. RESULTS: Aqueduct of Sylvius was the most common site of obstruction (19 patients) either secondary to focal, multifocal or tubular stenosis, adhesions, or secondary to extrinsic compression. Functional and anatomical correlation was analysed in 58 regions revealing strong correlation (ro = 0.8, p < .001). Functional anatomical mismatch was found in nine regions. Flow velocity measurements revealed diminished flow in most of the cases with obstruction at the aqueduct and normal velocity in cases with obstruction proximal to aqueductal level, while accelerated flow was seen in cases with infra-aqeuductal obstruction. CONCLUSION: Phase-contrast and 3D-DRIVE sequences are essential sequences in the diagnosis of hydrocephalus enabling perfect localization of the site of obstruction. Both sequences should be interpreted in conjunction to avoid false results. Velocity measurements through the aqueduct can help understand CSF hydrodynamics.


Asunto(s)
Hidrocefalia/diagnóstico por imagen , Imagenología Tridimensional/métodos , Imagen por Resonancia Magnética/métodos , Neuroimagen/métodos , Niño , Preescolar , Femenino , Humanos , Masculino , Estudios Retrospectivos
6.
Neuroradiology ; 59(11): 1155-1163, 2017 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-28864914

RESUMEN

PURPOSE: The purpose of this study was to explore brain abnormalities in nonketotic hyperglycinemia (NKH) using diffusion-weighted imaging (DWI) and when feasible, diffusion tensor imaging (DTI) and tractography. METHODS: Seven patients with confirmed diagnosis of NKH (8 days-2 years) underwent brain MRI. Conventional T1 and T2WI were acquired in all patients, DWI in six and DTI and tractography in two (4 months and 2 years). Measurements of fractional anisotropy (FA), radial diffusivity (RD), axial diffusivity (AD) and Trace from eight white matter regions were compared between the two patients and age-matched controls. Tractography of corpus callosum, superior longitudinal fasciculus and corticospinal tracts was performed with extraction of their FA and diffusivity indices. RESULTS: MRI showed nonspecific brain atrophy in three children. Corpus callosum atrophy was found as a part of these atrophic changes. Cerebellar vermian hypoplasia and supratentorial hydrocephalus were seen in one patient. The topographic distribution of diffusion restriction was different among patients. The affected white matter regions were not predominantly following the expected areas of myelination according to patients' age. Deep grey matter nuclei were affected in one patient. DTI revealed lower FA with higher RD in most of the measured white matter regions and tracts. These changes were more appreciated in the 2-year-old patient. However, Trace was higher in the 2-year-old patient and lower in the 4-month-old one. The extracted tracts were decreased in volume. CONCLUSION: DWI, DTI and tractography with FA and diffusivity measurements can give insights into white matter microstructural alterations that can occur in NKH.


Asunto(s)
Encefalopatías/diagnóstico por imagen , Encefalopatías/patología , Imagen de Difusión por Resonancia Magnética/métodos , Imagen de Difusión Tensora/métodos , Hiperglicinemia no Cetósica/diagnóstico por imagen , Hiperglicinemia no Cetósica/patología , Sustancia Blanca/patología , Anisotropía , Atrofia , Consanguinidad , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Factores de Riesgo
7.
Neuroradiology ; 59(7): 715-724, 2017 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-28580531

RESUMEN

PURPOSE: The aim of this study is to study the age, gender and lateral asymmetry-related white matter changes of long association tracts throughout late childhood and adolescence into adulthood using diffusion tensor tractography (DTT). METHODS: DTT was performed in 44 healthy subjects aged 7-45 years. Fractional anisotropy (FA), radial diffusivity (RD), axial diffusivity (AD), Trace, density and volume were calculated for long association tracts, namely the inferior fronto-occipital fasciculus (IFOF), inferior longitudinal fasciculus (ILF), uncinate fasciculus, superior longitudinal fasciculus (SLF) and its arcuate fibres. FA and diffusivity indices were correlated as function of age using Pearson correlation test. Comparison between males and females, and comparison between both hemispheres among all participants were also performed. A p value less than .01 was considered significant. RESULTS: The majority of the examined tracts (SLF and IFOF of both hemispheres, and the arcuate fasciculus, uncinate fasciculus, and ILF of the left hemisphere) followed a common pattern of metric changes with age. This pattern was characterized by significant FA increase accompanied by reduction in RD, Trace without significant AD changes. The right arcuate fasciculus showed similar pattern but without significant FA changes. The right uncinate and right ILF fasciculus demonstrated significant reduction in RD, Trace and AD, with and without significant FA increase, respectively. Left hemispheric dominance regarding the FA and diffusivity indices was demonstrated in uncinate fasciculus with no significant gender-related differences. CONCLUSION: Significant microstructural tract-specific maturation processes continue throughout late childhood into adulthood. These processes may represent stages in a cascade of age-related maturation in white matter microstructure.


Asunto(s)
Envejecimiento/fisiología , Imagen de Difusión Tensora/métodos , Sustancia Blanca/diagnóstico por imagen , Sustancia Blanca/fisiología , Adolescente , Adulto , Anisotropía , Mapeo Encefálico , Niño , Femenino , Humanos , Masculino , Valores de Referencia
8.
Pediatr Radiol ; 45(11): 1696-705, 2015 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26111870

RESUMEN

BACKGROUND: Glutaric aciduria type 1 is a rare neurometabolic disease with high morbidity. OBJECTIVE: To describe the MR imaging abnormalities in glutaric aciduria type 1 and to identify any association between the clinical and imaging features. MATERIALS AND METHODS: MRI scans of 29 children (mean age: 16.9 months) with confirmed diagnosis of glutaric aciduria type 1 were retrospectively reviewed. Gray matter and white matter scores were calculated based on a previously published pattern-recognition approach of assessing leukoencephalopathies. Hippocampal formation and opercular topography were assessed in relation to the known embryological basis. MRI scores were correlated with morbidity score. RESULTS: The most consistent MRI abnormality was widened operculum with dilatation of the subarachnoid spaces surrounding underdeveloped frontotemporal lobes. Incomplete hippocampal inversion was also seen. The globus pallidus was the most frequently involved gray matter structure (86%). In addition to the central tegmental tract, white matter abnormalities preferentially involved the central and periventricular regions. The morbidity score correlated with the gray matter abnormality score (P = 0.004). Patients with dystonia had higher gray matter and morbidity scores. CONCLUSION: Morbidity is significantly correlated with abnormality of gray matter, rather than white matter, whether secondary to acute encephalopathic crisis or insidious onset disease.


Asunto(s)
Errores Innatos del Metabolismo de los Aminoácidos/diagnóstico , Encefalopatías Metabólicas/diagnóstico , Encéfalo/patología , Glutaril-CoA Deshidrogenasa/deficiencia , Preescolar , Diagnóstico Diferencial , Femenino , Humanos , Lactante , Imagen por Resonancia Magnética/métodos , Masculino , Estadística como Asunto
9.
Pediatr Radiol ; 44(7): 831-8, 2014 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-24756250

RESUMEN

BACKGROUND: Anteriorly displaced anus is an anomaly that is debated with regard to its nomenclature, diagnosis and management. OBJECTIVE: To describe MRI anatomy of the anal canal in children with anteriorly displaced anus and its impact on the process of defecation. MATERIALS AND METHODS: We prospectively examined ten children (7 girls, 3 boys; age range 7 months to 8 years, mean 3 years) with anteriorly displaced anus between August 2009 and April 2012. Noncontrast MRI examinations were performed on a 1.5-T magnet. T1- and T2-weighted turbo spin-echo images were acquired in axial, sagittal and coronal planes of the pelvis. The anorectal angle and the relative hiatal distance were measured in mid-sagittal images, and compared with those of a control group using the Mann-Whitney test. RESULTS: In children with anteriorly displaced anus, no anatomical abnormality was depicted at the level of the proximal anal canal. However, the distal anal canal was displaced anteriorly, running out its external muscle cuff, which remained un-displaced at the usual site of the anus. This changes the orientation of the central axis of the anal canal by passing across instead of along the fibers of the longitudinal muscle coat. Children with anteriorly displaced anus had a more obtuse anorectal angle (mean 112.1°), which was significantly greater than that of the control group (mean 86.2°). CONCLUSION: MRI is a valuable tool in studying the anatomy of the anal canal in children with anteriorly displaced anus. The abnormal orientation of the longitudinal muscle across the anal canal can explain the obstructed defecation in these children. Based on this study, it might be of interest to use MRI in studying equivocal cases and children with unexplained constipation.


Asunto(s)
Canal Anal/anomalías , Canal Anal/patología , Imagen por Resonancia Magnética , Estudios de Casos y Controles , Niño , Preescolar , Estreñimiento/etiología , Femenino , Humanos , Lactante , Masculino , Músculo Liso/anomalías , Músculo Liso/patología , Estudios Prospectivos
10.
European J Pediatr Surg Rep ; 12(1): e26-e32, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38351952

RESUMEN

We present a simple surgical technique aiming to improve urine outflow through the common urogenital sinus in cloaca and facilitate drainage of existing hydrocolpos. The study included three cases of cloaca with associated hydrocolpos that were operated during the period 2022 through 2023. The patient is placed in the prone position for a standard posterior sagittal anorectoplasty. The distal rectal fistula is severed flush with the vagina/sinus leaving an open defect in the posterior wall of the vagina/sinus. The defect is then widened distally via a vertical incision (∼1 cm) through the posterior wall of the common urogenital sinus toward but not reaching the perineum. This vertical defect is then closed horizontally displacing the posterior vaginal wall downwards toward the perineum (posterior sinuplasty). The postoperative recovery was uneventful in the three cases. Adequate drainage of hydrocolpos was confirmed by imaging at follow-up, as well as improvement of upper urinary tract dilatation. In selected cases of cloaca, posterior sinuplasty is a simple procedure that can be applied during anorectoplasty to provide effective drainage of associated hydrocolpos.

11.
European J Pediatr Surg Rep ; 10(1): e93-e97, 2022 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-35911497

RESUMEN

"Cloaca" is a term used to describe an anomaly in the female where a single orifice is located in the perineum draining both urogenital and gastrointestinal tracts. Few reports used the same term "cloaca" to describe the counterpart anomaly in the male. We present two "male" cases of anorectal anomalies associated with significant penile deformity (caudally displaced penis) that were managed during the period between January 2010 and September 2021. Characteristically, both cases had a single "central" perineal orifice. The latter was located anterior to the predestined site of the normal anus and just beneath a caudally positioned hypospadiac phallus. The caudal displacement of the penis was strikingly obvious by the presence of severe form of penoscrotal transposition. Both cases were associated with a perineal swelling (hamartoma) just beside the central perineal orifice. The urethra was very short (like that in the female), besides the single perineal orifice, which makes the presentation very similar to cloacal anomalies.

12.
Glob Pediatr Health ; 9: 2333794X221085386, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35425848

RESUMEN

Objectives. This study was carried out to delineate the patients' characteristics and the imaging findings and their relation to some biochemical markers of 31 critically ill patients with MIS-C. Design. A retrospective cross-sectional study including all critically ill MIS-C patients admitted to the PICU from June 23rd to July 22nd, 2020. Results. Eighteen males and 13 females, with a median age of 9 years (interquartile range 6-11) presented mainly with fever (100%) and hypotension (100%). Abnormalities in the chest computed tomography were detected in 22 cases (71%). Consolidation and architecture distortion were detected in 58.1% of patients; bilateral lesions and lower lobe infiltrates, each, was evident in 64.5% of patients, while the peripheral distribution of lesions was seen in 71% of the cases. Pleural thickening and effusion, each, was found in 51.6% of the patients. In this small case series, the presence of high ferritin was significantly associated with the bilaterality of the lesions. Elevated C-reactive protein was associated with the peripheral distribution of the lesions. Thrombocytopenia and hypoalbuminemia were significantly correlated with the CT disease stage and CT severity score respectively. Conclusions. Although a few children in this group of MIS-C patients presented with respiratory manifestations, yet, most of them demonstrated significant radiological lung involvement, which necessitates a longer-term follow-up.

13.
European J Pediatr Surg Rep ; 9(1): e37-e40, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-34007781

RESUMEN

Infantile hemangiomas (IHs) are common vascular tumors. In most cases, a benign course with favorable outcome can be anticipated. IH typically present as cutaneous lesions either with a localized or diffuse segmental distribution. Segmental hemangiomas in the face may be associated with brain and cardiac anomalies (PHACES syndrome), whereas airway involvement has been reported to be associated with hemangiomas in the "beard" area. Multiple cutaneous hemangiomas may be associated with visceral hemangiomas (commonly in the liver). In this report, we present a new association where deep paravertebral hemangiomatous lesions were observed to be associated with cutaneous back hemangiomas in two consecutive cases.

14.
Int J Cardiovasc Imaging ; 37(2): 587-596, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-32909145

RESUMEN

The purpose of this study is to test the capability of a commercially available feature tracking-cardiac magnetic resonance (FT-CMR) strain analysis software module in differentiating between viable and non-viable myocardium in chronic ischemic patients. Thirty chronic ischemic patients and 10 healthy volunteers were enrolled. Cine images were used for peak circumferential and radial strains quantification using dedicated FT-CMR software. Global strain was compared between patients and controls. In patients, segmental strain was compared in viable and non-viable myocardium determined by late gadolinium enhancement (LGE); and in segments with wall abnormalities. Among 480 myocardial segments analyzed in patients, 76 segments were non-viable on LGE. The mean left ventricular ejection fraction (LVEF) of the patients (87% males, mean age 55 ± 12 years) was 40 ± 12% vs. 61 ± 5% for the controls (80% males, mean age 39 ± 11 years). Peak global circumferential strain (GCS) and global radial strain (GRS) were significantly impaired in patients compared to controls (-13.89 ± 4.12% vs. -19.84 ± 1.47%), p < 0.001 and (23.11 ± 6.59% vs. 31.72 ± 5.52%), p = 0.001. Segmental circumferential strain (SCS) and segmental radial strain (SRS) were significantly impaired in non-viable compared to viable segments (-9.47 ± 7.26% vs. -14.72 ± 7.5%), p < 0.001 and (15.67 ± 12.11% vs. 24.51 ± 16.22%), p < 0.001. Cut-off points of -9.36% for the SCS (AUC = 0.7, 95% CI = 0.63-0.77) and 19.5% for the SRS (AUC = 0.67, 95%CI = 0.61-0.73) were attained above which the segment is considered viable.SCS was able to discriminate between normokinetic, hypokinetic and akinetic segments (mean = 27.6 ± 17.13%, 18.66 ± 12.88% and 15.24 ± 10.70% respectively, p < 0.001). Circumferential and radial segmental strain analysis by FT-CMR was able to discriminate between viable and non-viable segments of the myocardium defined by LGE and between normokinetic, hypokinetic and akinetic segments, using routinely acquired cine images, and thus can provide a more objective metric for risk stratification in chronic ischemic patients.


Asunto(s)
Imagen por Resonancia Cinemagnética , Isquemia Miocárdica/diagnóstico por imagen , Miocardio/patología , Volumen Sistólico , Función Ventricular Izquierda , Adulto , Anciano , Enfermedad Crónica , Femenino , Humanos , Masculino , Persona de Mediana Edad , Isquemia Miocárdica/patología , Isquemia Miocárdica/fisiopatología , Valor Predictivo de las Pruebas , Pronóstico , Estudios Retrospectivos , Supervivencia Tisular
15.
J Pediatr Urol ; 17(3): 410.e1-410.e7, 2021 06.
Artículo en Inglés | MEDLINE | ID: mdl-33549475

RESUMEN

BACKGROUND: 'Persistent Cloaca' refers to one of the major groups of anorectal anomalies in the female when a single perineal orifice is located at the normal site of the urethra draining both urogenital and alimentary tracts. However, 'posterior cloaca' is a new term developed by Pena to describe a unique defect in females in which a common urogenital sinus is deviated posteriorly to open into normally located anorectum (type A) or in the perineum slightly anterior to the anus (type B). METHODS: The study included seven girls diagnosed as posterior cloaca. Their age at presentation ranged from 1 to 108 months (median 12 months). The main presentation was abnormal external genitalia, while two cases underwent colostomy (±vaginostomy) at birth. Surgical reconstruction varied according to the degree of deviation from normal anatomy. Partial urogenital sinus mobilization (PUM) was used to correct minor forms; while at the other end of the spectrum (absent urinary bladder), continent urinary diversion was performed. Due to the high incidence of renal and urological anomalies, regular follow up at paediatric nephrology/urology clinics was advised for affected cases. RESULTS: All seven cases had a common urogenital confluence characteristically deviated posteriorly. The degree of backward deviation of the common urogenital orifice was variable ranging from mild to severe posterior displacement. In six cases (85%), the common urogenital orifice was shifted backwards in the perineum approaching the anal verge (type B). In one case, the common urogenital orifice opened internally into the anorectum (type A). Major urinary tract anomalies were quite common (5 of 7 cases; 71%): absent urinary bladder (2 cases); single kidney (2 cases); crossed ectopic kidney (1 case); hydroureteronephrosis (2 cases). CONCLUSION: Posterior cloaca is a rare anomaly in the female essentially affecting the lower urogenital tract with a high incidence of associated renal anomalies. Management should be tailored according to the degree of developmental defect.


Asunto(s)
Malformaciones Anorrectales , Anomalías Urogenitales , Canal Anal/cirugía , Malformaciones Anorrectales/cirugía , Niño , Preescolar , Cloaca/cirugía , Femenino , Humanos , Lactante , Recién Nacido , Anomalías Urogenitales/diagnóstico por imagen , Anomalías Urogenitales/cirugía , Sistema Urogenital
16.
J Pediatr Surg ; 55(5): 977-984, 2020 May.
Artículo en Inglés | MEDLINE | ID: mdl-32037221

RESUMEN

PURPOSE: In this report, we aim to define the different degrees of structural abnormality affecting the female genitalia in cases of CAH by using the multiplanar capabilities and high soft tissue resolution of MRI. PATIENTS AND METHODS: The study included cases of CAH who were referred to our pediatric surgical facility for genital reconstruction during the period 2016 through 2019. We studied the pelvic MRI anatomy in cases of CAH while referring to clinical and operative findings. To set up a grading scale for the degree of virilization in cases of CAH, we included another two control groups of normal boys and girls representing the two ends of the spectrum. RESULTS: The study included 23 cases of CAH who underwent preoperative pelvic MRI examination. All cases had normal chromosomal analysis (46 XX). Their age ranged from 1 to 156 months at time of MRI examination (mean 42.4; median 25). The level of the lower end of the vagina was identified in midsagittal T2WI and confirmed in sequential axial cuts. Based on the level of the lower end of the vagina in relation to the pubic symphysis, we classified cases of CAH into either low or high types. Moreover, we could observe a correlation between the degree of vaginal descent and structural transformation of erectile tissue between both genders. CONCLUSION: MRI can have an important role in the evaluation of cases of CAH by displaying the severity of internal anomaly which is crucial for proper preoperative counseling. TYPE OF STUDY: Case control study. LEVEL OF EVIDENCE: Level III.


Asunto(s)
Hiperplasia Suprarrenal Congénita/diagnóstico por imagen , Genitales Femeninos/anomalías , Imagen por Resonancia Magnética , Adolescente , Hiperplasia Suprarrenal Congénita/cirugía , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Genitales Femeninos/diagnóstico por imagen , Genitales Femeninos/cirugía , Genitales Masculinos/anatomía & histología , Genitales Masculinos/diagnóstico por imagen , Humanos , Lactante , Masculino , Gravedad del Paciente , Pelvis/anatomía & histología , Pelvis/diagnóstico por imagen , Cuidados Preoperatorios , Valores de Referencia , Vagina/anomalías , Vagina/diagnóstico por imagen
17.
European J Pediatr Surg Rep ; 8(1): e90-e94, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-33335826

RESUMEN

Venous malformations represent a major sector of vascular anomalies. Most cases are asymptomatic or subclinical; however, large extensive lesions can cause severe disability and sometimes mortality. In this report, we present a successful case of sirolimus treatment in managing an extensive venous malformation in the pelvis of a 21-month-old boy who presented with life-threatening complications. With a history dating since the day 2 of life, the patient suffered from chronic bleeding due to scrotal skin ulcerations, in addition to recurrent attacks of severe bleeding per rectum necessitating hospital admission and blood transfusion (three attacks since the age of 7 months). Pelvic magnetic resonance image showed the typical findings of extensive venous malformation involving the pelvis, perineum, scrotum, and extending to the gluteal region. The lesion was seen totally encasing the anorectum with marked thickening of their walls almost occluding their lumen. Oral sirolimus (2 mg/m 2 ) was started with a target blood trough level of 5 to 10 ng/mL. Over a follow-up period of 5 months, there was obvious clinical improvement that included healing of skin lesions (scrotal ulcer) with complete re-epithelialization, absence of bleeding per rectum with improvement of constipation, and rise of hemoglobin level from 7.5 to 11.5 g/dL.

18.
J Pediatr Surg ; 55(6): 1145-1151, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-31718868

RESUMEN

Since we have started colonic replacement of the esophagus in children, several successive modifications have led to evolution of the surgical technique aiming to decrease complications and achieve the best functional outcome. Currently, our surgical group has reached a single standardized technique that has been applied to all cases included in this report. A colonic flap (including the transverse colon) is prepared based on the left colic vessels. The proximal end of the colonic flap is transferred upwards passing at first behind the stomach and then anteriorly through the lesser omentum to traverse the thoracic cavity via a previously prepared retrosternal tunnel. The proximal end of the colonic flap is anastomosed to the proximal esophagus in the neck (end to end anastomosis). Most specifically, the distal end of the colonic flap is anastomosed to the posterior wall of the body of the stomach in order to prevent regurgitation of gastric contents into the colonic flap. The retro-sternal colon bypass is among the successful options to replace the esophagus in children. Adding a posterior cologastric anastomosis to the procedure greatly prevents gastric regurgitation that can be responsible for short- and long-term complications. LEVEL OF EVIDENCE: This is a case series with no comparison group (level IV evidence).


Asunto(s)
Colon/cirugía , Atresia Esofágica/cirugía , Estenosis Esofágica/cirugía , Esófago/cirugía , Colgajos Quirúrgicos/cirugía , Anastomosis Quirúrgica/métodos , Niño , Preescolar , Estenosis Esofágica/etiología , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/prevención & control , Estómago/cirugía , Resultado del Tratamiento
19.
J Pediatr Surg ; 54(10): 2004-2011, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31072675

RESUMEN

PURPOSE: To study the detailed anatomy of cloacal anomalies using the multiplanar capabilities of MRI in addition to other available modalities. PATIENTS AND METHODS: The study was conducted on 27 cases of cloaca that were managed at our unit during the last 12 years. Preoperative assessment included conventional investigations and MRI studies. Endoscopic and operative findings were important for confirmation and completion of the whole picture. For better perception of the degree of deviation from the norm, we included another control group of girls who underwent pelvic MRI studies for causes other than anorectal anomalies. RESULTS: For practical reasons, we dissociated the cloacal complex into its two basic components to be described separately namely the anorectal anomaly and persistent urogenital sinus. The anorectal anomaly may be best described regarding two parameters: the position of the rectum and type of rectal communication with the urogenital tract. Persistent urogenital sinus anomalies were stratified based on referring the level of the urogenital confluence to the back of pubic symphysis. Three types could be identified (low, intermediate, and high) in which the mean length of the common channel was 6.4, 14.6, and 24 mm respectively with a significant statistical difference (Kruskal-Wallis chi-squared test). CONCLUSION: Applying MRI in the preoperative assessment of cases of cloaca has the advantage of demonstrating the real anatomy of the anomaly in multiple planes. This can improve our perception of the degree of deviation from the norm in these cases, which may be important during planning for surgical reconstruction. LEVEL OF EVIDENCE: This is a case control study (level III evidence).


Asunto(s)
Malformaciones Anorrectales/diagnóstico , Procedimientos Quirúrgicos del Sistema Digestivo/métodos , Imagen por Resonancia Magnética/métodos , Procedimientos de Cirugía Plástica/métodos , Radiografía/métodos , Recto/anomalías , Malformaciones Anorrectales/cirugía , Preescolar , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Recto/diagnóstico por imagen , Estudios Retrospectivos
20.
Insights Imaging ; 10(1): 3, 2019 Jan 28.
Artículo en Inglés | MEDLINE | ID: mdl-30689061

RESUMEN

CSF flow disorders are frequently encountered in children. The advent of MR technology with the emergence of new pulse sequences allowed better understanding of CSF flow dynamics. In this pictorial review, we aim to conduct a comprehensive review of the MR protocol used to study CSF flow disorders and to discuss the utility of each pulse sequence in the adopted protocol. We will focus on the key anatomical structures that should be examined to differentiate hydrocephalus form ventricular dilatation ex-vacuo. The MR features of obstructive and communicating hydrocephalus will be discussed, in addition to the manifestations of CSF disorders associated with posterior fossa malformations (Dandy-Walker malformation, Chiari, and Blake's pouch cyst). Moreover, the value of MRI in the assessment of patients following interventional procedures (ventriculoperitoneal shunt and third ventriculostomy) will be addressed.

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