Posterior Sinuplasty: A New Strategy for Managing Hydrocolpos in Cloaca-Case Series.

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.

Postnatal imaging of conjoined twins: a customized multimodality approach.

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.

Cloaca-Like Anomalies in the Male: A Report on Two Cases.

"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.

Clinical Characteristics and Pulmonary Computerized Imaging Findings of Critically Ill Egyptian Patients with Multisystem Inflammatory Syndrome in Children.

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.

Differential diagnostic features between congenital haemangioma, KHE, and congenital fibrosarcoma: a single-centre experience.

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.

Posterior Mediastinal and Cutaneous Back Hemangiomas in Infants: A New Association.

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.

Posterior cloaca: A urogenital rather than anorectal anomaly.

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.

Strain analysis using feature tracking cardiac magnetic resonance (FT-CMR) in the assessment of myocardial viability in chronic ischemic patients.

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.

Sirolimus: A Rescue Drug to Control Complications of Extensive Venous Malformation.

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.

Transformation of the female genitalia in congenital adrenal hyperplasia: MRI study.

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.

Correlation between diffusion tensor imaging measures and the reading and cognitive performance of Arabic readers: dyslexic children perspective.

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.

Colonic replacement of the esophagus: towards standardization of the technique.

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).

Currarino Triad: Importance of Preoperative Magnetic Resonance Imaging.

Currarino triad is a rare syndrome that may be occasionally encountered during managing cases of anorectal anomalies. The triad consists of anorectal anomaly, sacral bony defect, and a presacral mass. It may be familial or sporadic, with a reported female predominance. Identification of the characteristic notched sacrum (sacral scimitar) in plain X-ray (anteroposterior view) is considered the key for the diagnosis; however, not infrequently, this radiological sign is overlooked, especially with a small sacral defect. Excision of the presacral cyst is usually performed concomitantly during anorectoplasty. The prone position is the standard approach for posterior sagittal anorectoplasty (PSARP) in males; however, in females, the supine position can be used as an alternative (anterior sagittal anorectoplasty). In this case report, excision of the presacral cyst took place in two steps: the first excision during the PSARP procedure in the prone position, and a second operation in the supine lithotomy position to remove a residual component of the lesion that was missed during the primary operation. It was clear that the supine lithotomy position provided better access to explore the presacral space than the prone position, especially with a deeply located cyst as in our case. The role of magnetic resonance imaging (MRI) in the identification of the exact nature and extent of the lesion before surgery is crucial and should be performed in all cases.

Late Diagnosis of Complete Colonic and Rectal Duplication in a Girl with an Anorectal Malformation.

Complete colonic duplication is rare, and usually occurs as a part of the caudal duplication syndrome. In such cases, the diagnosis is clinically evident by the presence of two ani arranged side by side in the perineum, which is commonly associated with duplication of the external genitalia as well (double phallus or double vestibule). In this report, we present a special case of anorectal anomaly that was associated with complete tubular colonic duplication. The diagnosis was initially missed due to the uncommon sagittal arrangement of duplicated rectum: one rectum was ending externally into the perineum by rectoperineal fistula, while the other was hidden by its internal termination into the vagina. Our final diagnosis for this case was a variant of anorectal anomaly in the female, which was associated with complete colonic duplication. One colon (which was in the free mesenteric border) terminated anteriorly into the vagina as a part of a "short common channel" cloaca, while the other colon terminated by rectoperineal fistula. Although the anomaly seems to be rather complex and confusing, yet our case was associated with an excellent outcome due to the benign type of anorectal anomalies (rectoperineal fistula and "short common channel" cloaca) and absence of significant sacral dysplasia; in addition to adequate identification of the abnormal anatomy by appropriate investigations and the staged approach for surgical reconstruction.

The cloacal anomalies: Anatomical insights through a complex spectrum.

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).

The value of CSF flow studies in the management of CSF disorders in children: a pictorial review.

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.

Anatomical alterations following the 'PSARP' procedure: Correlating MRI findings with continence scores.

PURPOSE: To identify anatomical aberrations following PSARP procedure by using MRI, while correlating MRI findings to clinical outcome. PATIENTS AND METHODS: Between January 2014 and December 2017, we conducted our study on male patients with rectourethral fistula who underwent PSARP. Postoperative pelvic MRI studies were performed and correlated to their clinical continence scores (Rintala, and Krickenbeck classification). RESULTS: The study included 31 patients. Fourteen patients were retrieved from the hospital records and accepted to participate in the study; while the remaining 17 were collected from the fecal incontinence clinic. Their age ranged from 40 to 156 months (mean 83) We divided patients in the study into two groups according to their Rintala continence scores: (Group A) 15 patients with low scores (10 or less); and (Group B) 16 patients with higher scores (more than 10). We detected wider pelvic hiatus (hiatus/PC ratio) and more obtuse anorectal angle in group A than B. CONCLUSION: Several anatomical alterations can be detected by MRI following the PSARP procedure that include abnormalities in the striated muscle sphincter (attenuation/deficiency), deviated neorectum, and presence of excessive perirectal fat. A widened pelvic hiatus and/or obtuse anorectal angle may correlate with poor fecal continence in these patients. LEVEL OF EVIDENCE: This is a case control study (level III evidence).

Magnetic resonance imaging of developmental facial paresis: a spectrum of complex anomalies.

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.

Phase-contrast and three-dimensional driven equilibrium (3D-DRIVE) sequences in the assessment of paediatric obstructive hydrocephalus.

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.