scholarly journals Congenital Constriction Band Syndrome: Clinical Study of Three Cases in Togo

2021 ◽  
Vol 4 (3) ◽  
pp. 170-178
Author(s):  
Foli Agbeko ◽  
Fidèle Comlan Dossou ◽  
Mawouto Fiawoo ◽  
Elom Ounoo Takassi ◽  
Mawulolo Kossi Kato ◽  
...  
Keyword(s):  
Antenatal Diagnosis ◽  
Skin Lesions ◽  
Lower Limbs ◽  
Amniotic Band ◽  
Left Wrist ◽  
X Ray ◽  
Left Hand ◽  
First Case ◽  
Constriction Band ◽  
Constriction Band Syndrome

Introduction: Congenital constriction band syndrome (CCBS) or amniotic band syndrome is a complex set of congenital malformations, mainly affecting the limbs, but also the craniofacial and thoraco-abdominal region. The Patients: The purpose of this work is to describe the clinical and diagnostic aspects of CCBS in Togo in relation to 03 clinical cases. The Primary Diagnoses, Interventions, and Outcomes: The first case is characterized by an asymmetric bilateral superficial constriction groove of the lower limbs, pseudosyndactylias and synostosis of the tibia and fibula on X-ray. The second has a deep strangulation in the left arm with an amputation of the fingers associated with hypochromic skin lesions, poorly hemmed polylobal ear and skin growths. The latter characterized by persistent bridle, strangulation and ischemia downstream of that of the left wrist with postnatal amputation of the left hand. Conclusion: The strengthening of antenatal diagnosis, the introduction of genetic counselling and the establishment of a national malformations register should make it possible to improve the management of cases of amniotic flange disease.

scholarly journals Amniotic constriction band syndrome resulting in amputation caused by septate uterus: a case report

2020 ◽  
Vol 48 (9) ◽  
pp. 030006052094975
Author(s):  
Tian He ◽  
Hao Xu ◽  
Ping Sui ◽  
Xin Wang ◽  
Yujie Sun
Keyword(s):  
Clinical Evidence ◽  
Tissue Necrosis ◽  
Septate Uterus ◽  
Amniotic Band ◽  
Amniotic Band Syndrome ◽  
Constriction Band ◽  
Congenital Condition ◽  
Uterine Septum ◽  
The Right ◽  
Constriction Band Syndrome

Amniotic band syndrome is an unusual congenital condition characterized by manifestations of disfigurement and disablement. Patients with this condition may experience an array of clinical deformities, including constriction rings, digital defects, and even visceral defects. Although this disease has been identified for centuries, its etiology is still unknown. The present male patient was born by cesarean section at 34 weeks and 4 days of gestation. At birth, an amniotic band that encircled and constricted his right upper limb was observed. Four hours after the amniotic band was cut off, amputation was performed because the right limb remained insensate. The patient suffered from amniotic band syndrome and presented with a gangrenous limb leading to amputation at birth, which is extremely rare. Moreover, the patient’s mother suffered from a uterine septum, which has not been previously reported in this situation. Timely surgical treatment avoided further tissue necrosis threating the patient’s life. This rare case of amniotic band syndrome provides new clinical evidence for the “extrinsic theory”.

scholarly journals Two Rare Syndromic Syndactyly Cases in Neonates

2021 ◽  
Author(s):  
Pooja Prabhakar Kamath
Keyword(s):  
Genetic Counseling ◽  
Clinical Features ◽  
Lower Limbs ◽  
Alar Cartilage ◽  
Nasal Bridge ◽  
Left Hand ◽  
Rare Syndrome ◽  
First Case ◽  
Male Neonate ◽  
Otopalatodigital Syndrome

Abstract Introduction Isolated findings of syndactyly are benign. However, syndactyly can be associated with rare syndromes that need to be diagnosed for further management and for genetic counseling. Methods We present two cases of syndromic syndactyly in neonates. The first case is a 13-day-old female neonate with dysmorphic features. The neonate had clinical features of prominent forehead, hypertelorism, widely separated sagittal and metopic sutures, down-slanting eyes, low set ears, depressed nasal bridge, micrognathia, cleft palate, pectus excavatum, brachydactyly, and syndactyly of the second to fourth fingers bilaterally in upper limbs and in lower limbs.The second case is a 10-day-old male neonate with dysmorphism in the form of cleft alveolar ridge and palate, hyperplastic frenula, hypoplastic alar cartilage, syndactyly of the left hand, clinodactyly of the left lower limb toes, and amniotic bands. Discussion Case 1 was diagnosed as otopalatodigital syndrome because of the characteristic clinical features. This is a rare syndrome associated with syndactyly that often goes undiagnosed. Otopalatodigital syndrome spectrum disorders comprise of four phenotypically related conditions: otopalatodigital syndrome types 1 and 2, frontometaphyseal dysplasia, and Melnick–Needles syndrome. As it is associated with x-linked inheritance, its severity is more in males.Case 2 was diagnosed to have orofaciodigital syndrome because of the characteristic clinical features. It is another rare syndrome associated with syndactyly having abnormalities in the development of the oral cavity, face, and digits along with intellectual disability and renal system impairment. Conclusion There are fewer publications on these syndromes as they are rare and diagnosis is difficult. Recognizing these syndromes is key to further management and for genetic counseling.

DUPLICATED THUMB WITH ENORMOUS SOFT-TISSUE OEDEMA — PACIFIER TYPE OF THUMB DUPLICATION

Hand Surgery ◽  
2011 ◽  
Vol 16 (01) ◽  
pp. 91-93 ◽  
Author(s):  
Kensuke Ochi ◽  
Yukio Horiuchi ◽  
Shinichiro Takayama ◽  
Harukazu Saito
Keyword(s):  
Soft Tissue ◽  
Pathological Condition ◽  
Type Ii ◽  
X Ray ◽  
Left Hand ◽  
First Case ◽  
Thumb Duplication ◽  
Floating Type ◽  
Underlying Pathophysiology ◽  
Tissue Oedema

Here we presented the first case of pacifier type thumb duplication. A newborn Japanese girl with no family history had a duplicated thumb on her left hand. The duplicated thumb showed a very large, oedematous soft-tissue nubbin in its appearance and was resected on the fifth day after birth. X-ray showed hypoplastic phalanx bone, suggesting type II polydactyly. Histology of the resected thumb showed enormous oedema in its connective tissue with cartilaginous and neural elements. This case was quite similar to literary reported cases of pacifier polydactyly in post-axial polydactyly, and its pathological condition seemed to be distinctly different from floating type or rudimentary type thumb duplication. We considered this type of thumb duplication as pacifier type thumb duplication, rather than floating or rudimentary type, in order to understand its underlying pathophysiology and to avoid confusion in further discussions.

scholarly journals PSEUDO AINHUM - A PROSPECTIVE STUDY

2014 ◽  
Vol 21 (01) ◽  
pp. 044-048
Author(s):  
Rehan E Kibria
Keyword(s):  
Prospective Study ◽  
Lower Limbs ◽  
Successful Outcome ◽  
Penile Shaft ◽  
Amniotic Band ◽  
Constriction Band ◽  
Uncommon Condition ◽  
A Prospective Study ◽  
Study Setting ◽  
Rule Out

Background: Amniotic band syndrome also known as ADAM complex, amnioticband sequence, congenital constrictions, bands and Pseudo Ainhum is a congenital disordercaused by entrapment of fetal parts (usually a limb or digits) in fibrous amniotic bands while inutero. Syndrome which is featured by one or more characteristic fibrous bands that encircle,deepen, strangulate and amputate parts of the human body. It start in a fetus & continuesthroughout life if not checked and treated . It is not an uncommon condition in our country butunfortunately data remains scarce. Design: A prospective study. Setting: Pediatric & GeneralSurgical Department of CMH Multan. Period: June 2010 to June 2012. Materials and methods:Characteristic progressive constriction band was undertaken. The patients were examinedclinically and findings recorded. An X-ray to rule out underlying bone anomaly was done.Treatment required multiple staged procedures. Correction of the constriction ring was done byexcision and Z-plasty to prevent or alleviate lymphoedema, separation of distally fused digits andskin grafting of defect. Results: Fifteen patients comprising of ten male & five females wereseen. Age of presentation ranged from second week of life to 12 years with mean age of 14months. A total of twenty one limbs and one penile shaft were effected. These included two rightupper limbs, three left upper limbs, ten right lower limbs and six left lower limbs. Penile shaft ofone of the patients just short of glans was involved. Four main types of lesions were found whichcomprised of constriction rings, intra uterine amputations, acrosyndactyl and simple syndactyl.Conclusions: Pseudo Ainhum is a disease of unknown etiolgy & could cause morbidity in newborn. The syndrome & its complications are amenable to corrective surgery with good results.Early surgery is required for successful outcome.

Identification of Asbestos Fibres in a Water Sample by X-Ray Microanalysis

1975 ◽  
Vol 33 ◽  
pp. 274-275
Author(s):  
K. A. Brookes ◽  
D. Finbow ◽  
Madeleine Samuel
Keyword(s):  
Water Sample ◽  
Background Radiation ◽  
X Ray ◽  
Left Hand ◽  
Transmission Imaging ◽  
Different Types ◽  
Normal Specimen ◽  
Liquid Nitrogen Cooling ◽  
Normal Transmission ◽  
Additional Port

Investigation of the particulate matter contained in the water sample, revealed the presence of a number of different types and certain of these were selected for analysis.An A.E.I. Corinth electron microscope was modified to accept a Kevex Si (Li) detector. To allow for existing instruments to be readily modified, this was kept to a minimum. An additional port is machined in the specimen region to accept the detector, with the liquid nitrogen cooling dewar conveniently housed in the left hand cupboard adjacent to the microscope column. Since background radiation leads to loss in the sensitivity of the instrument, great care has been taken to reduce this effect by screening and manufacturing components that are near the specimen from material of low atomic number. To change from normal transmission imaging to X-ray analysis, the special 4-position specimen rod is inserted through the normal specimen airlock.

Congenital Syphilis Simulating Bone Neoplasm in 2-Month Old Infant – Case Report

2017 ◽  
Vol 2 (1) ◽  
Keyword(s):  
Case Report ◽  
Congenital Syphilis ◽  
Eosinophilic Granuloma ◽  
Osteolytic Lesion ◽  
Lower Limbs ◽  
Upper Body ◽  
Elective Cesarean Section ◽  
Distal Fibula ◽  
X Ray

Introduction: Congenital Syphilis (CS) occurs through the transplacental transmission of Treponema pallidum in inadequately treated or non-treated pregnant women, and is capable of severe consequences such as miscarriage, preterm birth, congenital disease and/or neonatal death. CS has been showing an increasing incidence worldwide, with an increase of 208% from 2009 to 2015 in Brazil. Case report: 2-month old infant receives care in emergency service due to edema of right lower limb with pain in mobilization. X-ray with osteolytic lesion in distal fibula. Infant was sent to the Pediatrics Oncology clinic. Perinatal data: 7 prenatal appointments, negative serology at 10 and 30 weeks of gestation. End of pregnancy tests were not examined and tests for mother’s hospital admission were not requested. Mother undergone elective cesarean section at 38 weeks without complications. During the pediatric oncologist appointment, patient showed erythematous-squamous lesions in neck and other scar-like lesions in upper body. A new X-ray of lower limbs showed lesions in right fibula with periosteal reaction associated with aggressive osteolytic lesion compromising distal diaphysis, with cortical bone rupture and signs of pathological fracture, suggestive of eosinophilic granuloma. She was hospitalized for a lesion biopsy. Laboratory tests: hematocrit: 23.1 / hemoglobin 7.7 / leukocytes 10,130 (without left deviation) / platelets 638,000 / Negative Cytomegalovirus IgG and IgM and Toxoplasmosis IgG and IgM / VDRL 1:128. Congenital syphilis diagnosis with skin lesions, bone alterations and anemia. Lumbar puncture: glucose 55 / total proteins 26 / VDRL non reagent / 13 leukocytes (8% neutrophils; 84% monomorphonuclear; 8% macrophages) and 160 erythrocytes / negative VDRL and culture. X-ray of other long bones, ophthalmological evaluation and abdominal ultrasound without alterations. Patient was hospitalized for 14 days for treatment with Ceftriaxone 100mg/kg/day, due to the lack of Crystalline Penicillin in the hospital. She is now under outpatient follow-up. Discussion: CS is responsible for high rates of morbidity and mortality. The ongoing increase of cases of this pathology reflects a severe health issue and indicates failures in policies for the prevention of sexually transmitted diseases, with inadequate follow-up of prenatal and maternity protocols.

Paraneoplastic pemphigus associated with rapidly progressing endophthalmitis caused by Morganella Morganii: A case report

2021 ◽  
pp. 112067212110334
Author(s):  
Abdullah I Almater ◽  
Mohammed M Abusayf ◽  
Saeed Alshahrani ◽  
Abdullah M Alfawaz ◽  
Hind M Alkatan ◽  
...  
Keyword(s):  
Positive Culture ◽  
Skin Lesions ◽  
Stevens Johnson Syndrome ◽  
Corneal Perforation ◽  
Morganella Morganii ◽  
Paraneoplastic Pemphigus ◽  
First Case ◽  
Johnson Syndrome ◽  
Acute Necrotizing ◽  
The Right

Introduction: The ocular presentation of paraneoplastic pemphigus (PNP) has rarely been reported in the literature. In this report, we describe a 61-year-old male presenting with eruptive skin lesions associated with underlying non-Hodgkin’s lymphoma who had rapid progressive corneal perforation with secondary endophthalmitis in the setting of PNP. Case description: A 61-year-old male presented to the emergency department complaining of skin eruption mimicking Stevens–Johnson syndrome, which was later found to be related to PNP. Initially, the patient complained of progressive ocular surface dryness in both eyes. Meanwhile, he developed mild pain in the right eye associated with blurry vision in both eyes and was managed with lubricants and topical antibiotics. A few days later, he was found to have corneal perforation with features suggestive of left endophthalmitis with possible early panophthalmitis. Intravenous antibiotic was administered, and primary evisceration of the left globe was performed. Histopathology revealed acute necrotizing keratitis and endophthalmitis. Vitreous analysis showed numerous gram-negative bacilli and a positive culture of Morganella morganii. The patient continued to be managed with frequent lubrications and punctual plugs in the fellow eye during the follow-up period. Conclusion: We describe the first case of endophthalmitis developing secondary to PNP-induced corneal melting and perforation. Anticipating unusual infectious sequelae in the setting of PNP might be warranted to actively detect and successfully manage dry eye disease before devastating complications develop.

Novel peroxosolvates of tetraalkylammonium halides: the first case of layers containing hydrogen-bonded peroxide molecules

CrystEngComm ◽  
2021 ◽  
Author(s):  
Mger A. Navasardyan ◽  
Stanislav Bezzubov ◽  
Alexander G. Medvedev ◽  
Petr V Prikhodchenko ◽  
Churakov Andrei
Keyword(s):  
Hydrogen Peroxide ◽  
X Ray ◽  
X Ray Crystallography ◽  
First Case ◽  
Tetraalkylammonium Halides ◽  
Hydrogen Bonded

Novel peroxosolvates of tetraalkylammonium halides Et4N+Cl–•2(H2O2) (1), Et4N+Br–•2(H2O2) (2), Me3(ClCH2CH2)N+Cl–•H2O2 (3) and Me3PhN+Cl–•H2O2 (4) were prepared from concentrated hydrogen peroxide and the corresponding structures were determined by X-ray crystallography. Structures...

scholarly journals Seventeen years of American cutaneous leishmaniasis in a Southern Brazilian municipality

2012 ◽  
Vol 54 (4) ◽  
pp. 215-218 ◽  
Author(s):  
Wilsandrei Cella ◽  
Simone Cristina Castanho Sabaini de Melo ◽  
Cátia Millene Dell Agnolo ◽  
Sandra Marisa Pelloso ◽  
Thaís Gomes Verzignassi Silveira ◽  
...  
Keyword(s):  
Cutaneous Leishmaniasis ◽  
Rural Area ◽  
Prevalence Rate ◽  
Endemic Area ◽  
Skin Lesions ◽  
Lower Limbs ◽  
Age Group ◽  
American Cutaneous Leishmaniasis ◽  
Incomplete Filling ◽  
Residence Location

We reviewed the records of 151 patients diagnosed with American cutaneous leishmaniasis (ACL) from 1993 to 2009 in the municipality of Japura, Paraná, Brazil. Gender, age, occupation, place of residence, location of lesions, type and number of lesions were analyzed. The prevalence rate of ACL was 11.5/10,000 hab, of which 84.7% were male, 58.3% lived in rural area and 49.0% were farmers. The most frequent age group was between 30 to 39 years (26.6%). Skin lesions occurred in 92.7% of the patients with predominance in the lower limbs (23.9%) and 49.1% of the records did not include the number of lesions location due to incomplete filling. A single ulceration was present in 44.4%. Japurá is an endemic area for ACL, requiring public actions and preventive education.

Sign in / Sign up

Export Citation Format

Share Document