scholarly journals Clinical Quiz—A Rare Case of Anal Canal Duplication in the Context of Currarino Syndrome

2021 ◽  
Vol 09 (01) ◽  
pp. e68-e71
Author(s):  
Sean Jared Connor ◽  
Giulia Brisighelli ◽  
Nirav Patel ◽  
Marc A. Levitt
Keyword(s):  
Anal Canal ◽  
Rare Case ◽  
Multidisciplinary Approach ◽  
Rare Condition ◽  
Management Plan ◽  
Clinical Quiz ◽  
Presacral Mass ◽  
Currarino Syndrome ◽  
Sacral Defect ◽  
Anal Canal Duplication

AbstractCurrarino syndrome (CS) is a rare condition that presents with any combination of a sacral defect, a presacral mass, and an anorectal malformation. This collection, referred to as Currarino's triad, may not necessarily present as all three abnormalities in the diagnosis of the syndrome. Anal canal duplication (ACD) is an even rarer occurrence. We present a case that lies on the CS spectrum with an associated ACD and discuss a complex surgical challenge that necessitated a customized management plan, devised through a multidisciplinary approach.

Currarino Syndroma, initially misdiagnosed and treated like megacolon congenitum

Open Medicine ◽  
2011 ◽  
Vol 6 (6) ◽  
pp. 723-726
Author(s):  
Ivona Djordjevic ◽  
Danica Jovanovic ◽  
Blagoje Grujic
Keyword(s):  
Molecular Genetic ◽  
Genetic Diagnosis ◽  
Hereditary Disease ◽  
Postoperative Recovery ◽  
Presacral Mass ◽  
Currarino Syndrome ◽  
Radiologic Evaluation ◽  
Definitive Operation ◽  
Megacolon Congenitum ◽  
Sacral Defect

AbstractCurrarino syndrome was first described as a triad by Guido Currarino, an American radiologist in 1981. It is an autosomal dominant hereditary disease known by the triad of anorectal stenosis, anterior sacral defect, and a presacral mass that is most often an anterior sacral meningocele.We represent a 3 year boy with refractory constipation from birth, which had been wrongly diagnosed and treated as Hirschsprung’s disease since early childhood. The patient underwent urgent colostomy because of water intoxication due to bowel irrigation, and following investigations (CT, MRI) revealed anorectal anomaly and presacral mass compatible with Curarrino’s syndrome.Definitive operation was done with perineal approach, posterior sagittal anorectoplasty, with interval colostomy closure 2 months after perineal operation. An uncomplicated postoperative recovery ensued. The patient’s bowel habits successfully returned to normal soon after the operation. A multidisciplinary diagnostic and therapeutic protocol is presented. Main points are rectal examination, sacrum x-Ray, molecular genetic diagnosis, radiologic evaluation of every member of Currarino syndrome families, CT scan, magnetic resonance (MR) evaluation of patient spine and pelvis and suction rectal biopsies. If the mass is a meningocele, colostomy and neurosurgical exploration should precede anoplasty due to the risk of meningitis.

scholarly journals Variants of Currarino Syndrome: Embryological Association and Review of Pertinent Literature

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Rahul Gupta ◽  
Shyam Bihari Sharma ◽  
Praveen Mathur ◽  
Ram Babu Goyal
Keyword(s):  
Sacrococcygeal Teratoma ◽  
Presacral Mass ◽  
Wound Sepsis ◽  
Causative Gene ◽  
Currarino Syndrome ◽  
Cloacal Malformation ◽  
First Case ◽  
Sacral Defect ◽  
Malformation Complex ◽  
Sigmoid Loop Colostomy

Currarino syndrome is a triad of sacral defect, anorectal malformation and a presacral mass. The diagnosis is usually made late in childhood and about 50% of cases are familial with autosomal dominant inheritance. We present two neonates (one with vestibular fistula, and another with cloacal malformation) with the features consistent with Currarino syndrome, but with Altman’s type II sacrococcygeal teratoma, that is, presacral mass having an external sacrococcygeal component also. We believe that this triad should be considered a variant of Currarino syndrome. In first case, excision of the mass along with coccyx, followed by primary Posterior Sagittal AnoRectoPlasty was performed in the same setting. The patient succumbed to death due to septicemia as a result of wound sepsis. Learning from the previous experience, we decided to do a diverting sigmoid loop colostomy followed by posterior sagittal excision of the mass along with coccyx, in same sitting in the second case. There was no recurrence. Though HLXB9 has been identified as the major causative gene in Currarino syndrome, exact pathogenesis is still unclear. We herein highlight the significance of this variant of Currarino syndrome and propose a theory on the basis of an embryological association between the malformation complex.

Currarino syndrome and spinal dysraphism

2014 ◽  
Vol 13 (6) ◽  
pp. 685-689 ◽  
Author(s):  
Matthew J. Kole ◽  
Jared S. Fridley ◽  
Andrew Jea ◽  
Robert J. Bollo
Keyword(s):  
Imperforate Anus ◽  
Congenital Anomalies ◽  
Rare Case ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Tethered Cord Syndrome ◽  
Presacral Mass ◽  
Occult Spinal Dysraphism ◽  
Spinal Lipoma ◽  
Currarino Syndrome

Currarino syndrome is a rare constellation of congenital anomalies characterized by the triad of sacral dysgenesis, presacral mass, and anorectal malformation. It is frequently associated with other congenital anomalies, often including occult spinal dysraphism. Mutations in the MNX1 gene are identified in the majority of cases. The authors report a rare case of Currarino syndrome in an infant with tethered cord syndrome and a dorsal lipomyelomeningocele continuous with a presacral intradural spinal lipoma, in addition to an imperforate anus and a scimitar sacrum. They review the literature to highlight patterns of occult spinal dysraphism in patients with Currarino syndrome and their relationship to tethered cord syndrome. Approximately 60% of the patients with Currarino syndrome reported in the literature have an occult spinal dysraphism. Published studies suggest that the risk of tethered cord syndrome may be higher among patients with a lipoma and lower among those with a teratoma or anterior meningocele.

scholarly journals The Controversy of Surgical Intervention for Anal Canal Duplication in Children

2020 ◽  
Vol 36 (6) ◽  
Author(s):  
Fatih Akova ◽  
Serdar Altinay ◽  
Emrah Aydin
Keyword(s):  
Anal Canal ◽  
Surgical Intervention ◽  
Retrospective Chart Review ◽  
Rare Condition ◽  
Primary Outcome Measure ◽  
Cystic Mass ◽  
Creative Commons ◽  
Definition Of ◽  
Tail Gut Cyst ◽  
Anal Canal Duplication

Objective: Since the first definition of anal canal little has been discovered about the etiology of this rare condition. We present four asymptomatic cases of anal canal duplication with diverse clinical and surgical findings. Methods: A retrospective chart review was performed on four infants presenting with asymptomatic anal canal duplication, born between 2014 and 2016. Clinical characteristics and pathologic findings of patients either by radiological imaging or pathology were evaluated. The primary outcome measure was the complications. Results: All patients were followed-up with physical examination and ultrasound for a mean of 3.5±1.0 years, lastly seen at the beginning of 2018. The female to male ratio was 3:1. Duplicate anal canal length varied between 12-20mm, and two of the four patients had a presacral cystic mass confirmed as a tail gut cyst following surgery. At follow-up, none of the patients had developed symptoms related to anal canal duplication, regardless of whether they had surgical intervention. Conclusion: Though surgical management is the preferred treatment for anal canal duplication, it seems that patients who do not undergo surgery might remain free of symptoms, suggesting that surgical intervention may be unnecessary. doi: https://doi.org/10.12669/pjms.36.6.1832 How to cite this:Akova F, Altinay S, Aydin E. The Controversy of Surgical Intervention for Anal Canal Duplication in Children. Pak J Med Sci. 2020;36(6):---------. doi: https://doi.org/10.12669/pjms.36.6.1832 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

scholarly journals Cerebral arteriovenous malformations during pregnancy: a management dilemma

2019 ◽  
Vol 12 (11) ◽  
pp. e228759
Author(s):  
Rubina Sohail ◽  
Qasim Bashir ◽  
Shahlla Kanwal ◽  
Maryam Iqbal Ali
Keyword(s):  
Arteriovenous Malformations ◽  
Intracranial Haemorrhage ◽  
Multidisciplinary Approach ◽  
Rare Condition ◽  
Management Plan ◽  
Maternal Condition ◽  
Cerebral Arteriovenous Malformations ◽  
Risk Of Bleeding ◽  
Brain Avm ◽  
The Family

The rupture of a brain cranial arteriovenous malformation (bAVM) has been associated with pregnancy; however, due to scarcity of data about this rare condition, management still remains a dilemma both for obstetricians and neurophysicians. The management plan is decided after weighing the benefits of bAVM treatment against the risk of bleeding in pregnancy. There is consensus on deciding the treatment of ruptured brain AVM during pregnancy based on neurological and not obstetrical indications. The management is decided using a multidisciplinary approach.We report the case of a primigravida who presented at 15 weeks of gestation with intracranial haemorrhage secondary to ruptured bAVM, which was managed by glue embolisation. However, she presented again after 9 days in a moribund condition with fever, vomiting and malaise. The family was very concerned about the patient. The multidisciplinary team after evaluation of patient decided for hysterotomy based on the deteriorating maternal condition.

Anal canal duplication in children: a monocentric experience of 12 cases

2021 ◽  
Author(s):  
Antoine Ailhaud ◽  
Oyekashopefoluw Alao ◽  
Eva Sole Cruz ◽  
Romain Faguet ◽  
Pierre-Louis Verot ◽  
...  
Keyword(s):  
Anal Canal ◽  
Anal Canal Duplication

scholarly journals Hypertrophy of the abductor hallucis muscle: A case report and review of the literature

2017 ◽  
Vol 5 ◽  
pp. 2050313X1772763 ◽  
Author(s):  
Toshinori Kurashige
Keyword(s):  
Rare Case ◽  
Muscle Hypertrophy ◽  
Pathological Condition ◽  
Rare Condition ◽  
Review Of The Literature ◽  
Resonance Imaging ◽  
Abductor Hallucis ◽  
Medial Plantar Nerve ◽  
Abductor Hallucis Muscle ◽  
Entrapment Syndromes

Objectives: Muscle hypertrophy is a relatively rare condition that may cause nerve entrapment syndromes. We report the case of a 14-year-old girl with unilateral hypertrophy of the abductor hallucis muscle with entrapment of the medial plantar nerve and review the literature. Methods: Computed tomography and magnetic resonance imaging revealed unilateral hypertrophy of the abductor hallucis muscle. Results: Two injections of steroid and lidocaine at the point of tenderness resulted in resolution of the pain. Conclusions: We report a rare case of hypertrophy of the abductor hallucis muscle considered with entrapment of the medial plantar nerve. Treatment of this condition should be selected according to the pathological condition of each patient.

Anal canal duplication in childhood

1996 ◽  
Vol 11 (8) ◽  
pp. 577-579 ◽  
Author(s):  
Y. Hamada ◽  
M. Sato ◽  
K. Hioki
Keyword(s):  
Anal Canal ◽  
Anal Canal Duplication

scholarly journals Complex-Compound Odontoma: A Rare Clinical Presentation

2018 ◽  
pp. 39-44
Author(s):  
Damla Torul DDS, PhD ◽  
Metehan Keskin DDS ◽  
Seda Gun DDS, PhD ◽  
Didem Odabasi DDS, PhD
Keyword(s):  
Early Detection ◽  
Complex Compound ◽  
Rare Case ◽  
Multidisciplinary Approach ◽  
Clinical Presentation ◽  
Odontogenic Tumor ◽  
Compound Odontoma

Odontomas can be detected as complex or compound variants and they rarely show the histologic characteristics of both types together. The tumor commonly associated with malocclusion, eruption disturbances and pathological anomalies, but they seldom cause bony expansion. Early detection and management of odontoma with multidisciplinary approach pose an important role to prevent disturbances associated with this common odontogenic tumor. Here we report a rare case of an odontoma which show the features of both complex and compound types and also cause bony expansion, eruption failure in an 8-year-old boy.

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