scholarly journals Clinical and Imaging Features of a Congenital Midline Cervical Cleft in a Neonate: A Rare Anomaly

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Rachelle Goldfisher ◽  
Pritish Bawa ◽  
Zachary Ibrahim ◽  
John Amodio
Keyword(s):  
Congenital Anomaly ◽  
Pediatric Surgery ◽  
Clinical Presentation ◽  
Imaging Features ◽  
Rare Congenital Anomaly ◽  
Rare Anomaly ◽  
Work Up ◽  
Midline Cervical Cleft

Congenital midline cervical cleft (CMCC) is a rare congenital anomaly. CMCC and its complications and treatment have been well described in ENT, dermatology, and pediatric surgery literature. However, to our knowledge, the imaging work-up has not been reported in the literature thus far. We present a case of CMCC in a neonate with description of clinical presentation and imaging features.

scholarly journals Triphalangeal thumb: clinical features and treatment

2018 ◽  
Vol 44 (1) ◽  
pp. 69-79 ◽  
Author(s):  
Steven E. R. Hovius ◽  
Jacob W. P. Potuijt ◽  
Christianne A. van Nieuwenhoven
Keyword(s):  
Congenital Anomaly ◽  
Surgical Treatment ◽  
Ligament Reconstruction ◽  
Soft Tissues ◽  
Clinical Presentation ◽  
Rare Congenital Anomaly ◽  
First Ray ◽  
Triphalangeal Thumb ◽  
Delta Phalanx ◽  
Complex Cases

Triphalangeal thumb is a rare congenital anomaly in which the thumb has three phalanges. Clinical presentation of triphalangeal thumb can vary considerably and can be present in both hands or unilateral. The thumb can be long with a finger-like appearance. The presence of clinodactyly depends on the shape of the extra phalanx varying from wedge-shaped to rectangular. Various joints, ligaments, muscles, and tendons of the first ray can be hypoplastic or absent, with varying degrees of stiffness or instability. The aim of surgical treatment is to reconstruct or correct the anatomic anomalies to obtain greater function and a more acceptable appearance. In our series, operations varied from removal of the delta phalanx with ligament reconstruction to multiple osteotomies and rebalancing of soft tissues. Results in these often complex cases can be rewarding if the surgeon has sufficient knowledge of the underlying anatomic differences. This review summarizes our current concepts of presentation and management of the triphalangeal thumb.

Midline Cervical Cleft: A Rare Congenital Anomaly

2010 ◽  
Vol 119 (11) ◽  
pp. 786-790
Author(s):  
Gayathri Mandya Renukaswamy ◽  
Marlene A. Soma ◽  
Benjamin E. J. Hartley
Keyword(s):  
Congenital Anomaly ◽  
Differential Diagnosis ◽  
Surgical Excision ◽  
Soft Tissue Defect ◽  
Suprasternal Notch ◽  
Anterior Neck ◽  
Street Hospital ◽  
Rare Congenital Anomaly ◽  
Great Ormond Street Hospital ◽  
Midline Cervical Cleft

Objectives: A midline cervical cleft (MCC) is a rare congenital anomaly due to failure of fusion of the first and second branchial arches during embryogenesis. It may present as a midline defect of the anterior neck skin with a skin projection or sinus, or as a subcutaneous fibrous cord. This report evaluates the clinical features and surgical management of an MCC. Methods: We analyzed a series of 4 patients with an MCC successfully treated at Great Ormond Street Hospital for Children in London. Results: Three male patients and 1 female patient between 4 and 11 months of age were found to have an MCC. Each patient presented with an erythematous, fibrous band of tissue extending between the chin and the suprasternal notch. Treatment comprised surgical excision of the lesion and Z-plasty repair. We present the embryology, common clinical presentation, investigations, differential diagnosis, and histology, along with a literature review, of this uncommon malformation of the anterior neck. Conclusions: An MCC is a differential diagnosis to consider when assessing a child with a midline cervical lesion. Early surgical excision with Z-plasty repair of the soft tissue defect is the treatment of choice to prevent long-term complications.

scholarly journals Uncomplicated bifid Meckle’s diverticulum mimicking recurrent appendicitis

2018 ◽  
Vol 7 (1) ◽  
pp. 1094-1096
Author(s):  
N Ravishankar ◽  
V Thulasi
Keyword(s):  
Congenital Anomaly ◽  
Acute Appendicitis ◽  
Meckel’S Diverticulum ◽  
Pancreatic Tissue ◽  
Meckel's Diverticulum ◽  
Distal Ileum ◽  
Rare Congenital Anomaly ◽  
Rare Anomaly ◽  
Recurrent Appendicitis ◽  
Meckel’S Diverticulitis

Meckel’s diverticulum is the rare congenital anomaly in children. When inflamed it can mimic acute appendicitis. The distal end of the Meckel’s diverticulum is usually rounded and narrow. We present a case of partially bifid Meckel’s diverticulum in a young boy who presented with features of recurrent appendicitis. During appendectomy, a bifid Meckle’s diverticulum was found in therein the distal ileum. It was excised with V shaped ileal wall. Histopathology showed features of Meckel’s diverticulum without any Gastric or pancreatic tissue in mucosa. Clinicians should be wary of a bifid meckel’s diverticulum as a very rare anomaly that can be symptomatic mimicking appendicitis.Keywords: Bifid, Meckel’s, Diverticulitis

scholarly journals Malrotation presenting beyond infancy: a clinical study

2018 ◽  
Vol 5 (10) ◽  
pp. 3288
Author(s):  
Anil Balraj T. ◽  
S. Srinivas ◽  
K. Ramesh Reddy ◽  
Manisha Reddy G. ◽  
Anusiri Inugala
Keyword(s):  
Congenital Anomaly ◽  
Pediatric Surgery ◽  
Clinical Presentation ◽  
Neonatal Period ◽  
Bowel Ischemia ◽  
Poor General Condition ◽  
Younger Patients ◽  
Small Intestinal ◽  
High Index Of Suspicion ◽  
And Child Health

Background: Intestinal malrotation is a congenital anomaly that results from abnormal or incomplete rotation and fixation of midgut during embryonic development. This study was done at Niloufer Hospital and Institute for Women and Child Health, Hyderabad with an aim of evaluating the clinical presentation, diagnosis, management and outcome of malrotation presenting beyond 1 year of lifeMethods: Overall 50 cases of malrotation presented to the department of pediatric surgery over a period of 2 year (April 2015 to April 2017). 12 patients of these 50 (12/50) whose age was above 1 year i.e. who were beyond infancy were studied in this studyResults: Out of the 12 patients, 6 were female and 6 were male. Age of patients ranged from 15 months to 13 years. Younger patients below 2 years commonly presented with typical symptoms of bilious vomiting and patients above 2 years of age presented with more varied and vague symptoms. 1/12 of patients presented with a poor general condition and succumbed during the course of treatmentConclusion: Small intestinal obstruction due to malrotation is relatively common in neonatal period. Malrotation beyond infancy is an uncommon diagnosis. Malrotation in grown up children usually is not suspected due to varied symptoms. High index of suspicion is needed as early intervention and treatment in the form of Ladds procedure can prevent any catastrophic events like volvulus and bowel ischemia.

scholarly journals Bilateral congenital renal arteriovenous malformation: A rare entity with uncommon presentation

2020 ◽  
Vol 8 (1) ◽  
Author(s):  
Radhiana H ◽  
Mohd Shafie A ◽  
Mohd Ariff MA
Keyword(s):  
Congenital Anomaly ◽  
Arteriovenous Malformation ◽  
Urinary System ◽  
Rare Entity ◽  
Rare Congenital Anomaly ◽  
Uncommon Presentation ◽  
Renal Arteriovenous Malformation ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up

Renal arteriovenous malformation (AVM) is a rare congenital anomaly of the urinary system. We present a patient with bilateral renal AVMs who presented with back pain and microscopic hematuria. This case highlights the importance of careful diagnostic work-up in the evaluation of upper tract hematuria.

scholarly journals Įgimta dvylikapirštės žarnos obstrukcija dėl preduodeninės vartų venos ir atvirkštinės skrandžio ir blužnies padėties

2010 ◽  
Vol 8 (1) ◽  
pp. 0-0
Author(s):  
Pranas Gurskas ◽  
Kęstutis Trainavičius ◽  
Aidas Ivanauskas ◽  
Arūnas Strumila
Keyword(s):  
Congenital Anomaly ◽  
Portal Vein ◽  
Pediatric Surgery ◽  
World Literature ◽  
Duodenal Obstruction ◽  
Rare Congenital Anomaly ◽  
Preduodenal Portal Vein ◽  
Congenital Duodenal Obstruction ◽  
The World ◽  
Hospital Center

Pranas Gurskas, Kęstutis Trainavičius, Aidas Ivanauskas, Arūnas StrumilaVilniaus universiteto Vaikų ligų klinikos Vaikų chirurgijos centras,Santariškių g. 7, LT-2600 VilniusEl paštas: [email protected] Įvadas / tikslasPreduodeninė vartų vena (PDVV) yra labai reta įgimta anomalija. Literatūroje aprašyta per 80 atvejų. Preduodeninė vartų vena dažniausiai nesukelia jokių klinikinių simptomų. Tik nedaugeliui pacientų ji gali būti dvylikapirštės žarnos nepraeinamumo priežastis. Tyrimo tikslas – aprašyti labai retą, įgimtą dvylikapirštės žarnos obstrukcijos priežastį ir pasidalyti šios anomalijos diagnostikos ir gydymo patirtimi. Ligoniai ir metodaiMes gydėme du pacientus dėl dvylikapirštės žarnos nepraeinamumo, kurio priežastis – preduodeninė vartų vena kartu su atvirkštine skrandžio ir blužnies padėtimi, nevisiškas žarnyno posūkis. RezultataiNaujagimis ir 1 metų 4 mėnesių mergaitė buvo operuoti dėl preduodeninės vartų venos, sukėlusios dvylikapirštės žarnos obstrukciją. Abiem vaikams buvo atliktos duodenoduodenostomijos. Pooperacinė abiejų ligonių eiga buvo sklandi. Vaikai pradėjo normaliai valgyti, nustojo vemti, ėmė priaugti svorio. IšvadosDuodenoduodenostomija yra veiksminga operacija gydant dvylikapirštės žarnos obstrukciją dėl preduodeninės vartų venos. Reikšminiai žodžiai: įgimtas dvylikapirštės žarnos nepraeinamumas, preduodeninė vartų vena Congenital duodenal obstruction due to preduodenal portal vein, associated with situs inversus of stomach and spleen Pranas Gurskas, Kęstutis Trainavičius, Aidas Ivanauskas, Arūnas StrumilaVilnius University Children’s Hospital, Center of Pediatric Surgery,Santariškių Str. 7, LT-2600 Vilnius, LithuaniaE-mail: [email protected] Background / objectivePreduodenal portal vein (PDPV) is a rare congenital anomaly. There are only 80 cases described in the world literature. The presence of this anomaly is rarely recognized as an emergency condition, and it can manifest as duodenal obstruction in very few patients. The aim of our study was to descibe this uncommon reason for duodenal obstruction and to share experience in recognizing and treating this pathology. Patients and methodsAuthors present two cases of duodenal obstruction caused by the preduodenal portal vein and associated with the inverted gastric and spleen position and malrotation of the gut. ResultsA male newborn and a 1 yr 4 mon old girl were operated on due to duodenal obstruction caused by the preduodenal portal vein. Both patients received a duodenoduodenostomy procedure. The recovery was uneventful. The patients became free of symptoms, returned to oral feeds, started to thrive. ConclusionsDuodenoduodenostomy is a safe and effective procedure in treating duodenal obstruction caused by the preduodenal portalvein. Key words: duodenal obstruction, preduodenal portal vein

scholarly journals Hypoplastic Coronary Artery Disease Presenting with Ventricular Fibrillation Cardiac Arrest

2021 ◽  
Author(s):  
Abra Guo ◽  
Hooman Bakhshi ◽  
James O'Hara ◽  
Leonard Genovese ◽  
Adam Fein ◽  
...  
Keyword(s):  
Heart Failure ◽  
Congenital Anomaly ◽  
Coronary Artery Disease ◽  
Cardiac Arrest ◽  
Coronary Artery ◽  
Cardiac Death ◽  
Rare Congenital Anomaly ◽  
Cardioverter Defibrillator ◽  
Artery Disease ◽  
Work Up

Hypoplastic coronary artery disease is a rare congenital anomaly that may present with ischaemic heart disease, heart failure or sudden cardiac death (SCD). We describe a case of cardiac arrest in a healthy young man. Work-up revealed a hypoplastic left anterior descending artery. The patient underwent cardioverter-defibrillator implantation for secondary prevention.

Midline Cervical Cleft: A Rare Congenital Anomaly

2009 ◽  
Vol 118 (11) ◽  
pp. 786-790 ◽  
Author(s):  
Gayathri Mandya Renukaswamy ◽  
Marlene A. Soma ◽  
Benjamin E. J. Hartley
Keyword(s):  
Congenital Anomaly ◽  
Rare Congenital Anomaly ◽  
Midline Cervical Cleft

Agenesis of the venous duct: two cases of extrahepatic drainage of the umbilical vein and extrahepatic portosystemic shunt with a review of the literature

2014 ◽  
Vol 25 (2) ◽  
pp. 208-217 ◽  
Author(s):  
Rohit S. Loomba ◽  
Michele Frommelt ◽  
David Moe ◽  
Amanda J. Shillingford
Keyword(s):  
Congenital Anomaly ◽  
Clinical Presentation ◽  
Umbilical Vein ◽  
Portosystemic Shunt ◽  
Review Of The Literature ◽  
Rare Congenital Anomaly ◽  
Venous Circulation

AbstractAgenesis of the venous duct is a rare congenital anomaly resulting in abnormal drainage of the umbilical vein into the foetal venous circulation. The clinical presentation and prognosis is variable, and may depend on the specific drainage pathways of the umbilical vein. We present two foetuses with agenesis of the venous duct, both associated with a postnatal portosystemic shunt, but with markedly different postnatal clinical courses. We also review all previously reported cases to better characterise this foetal disorder and the prognosis.

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