Extensive Facial Clefting in a Patient with Goltz Syndrome: Multidisciplinary Treatment of a Previously Unreported Association

2002 ◽  
Vol 39 (4) ◽  
pp. 469-473 ◽  
Author(s):  
Jeffrey A. Ascherman ◽  
Sean L. Knowles ◽  
Kenneth C. Troutman
Keyword(s):  
Musculoskeletal System ◽  
Multidisciplinary Approach ◽  
Multidisciplinary Treatment ◽  
Severe Form ◽  
Female Infant ◽  
Craniofacial Abnormalities ◽  
Multisystem Disorder ◽  
Facial Region ◽  
Facial Cleft ◽  
Goltz Syndrome

Objective Goltz syndrome is a rare, X-linked dominant, multisystem disorder found almost exclusively in female patients. Although the cutaneous features predominate in most reports, characteristic abnormalities are also frequently present in the musculoskeletal system and facial region. We report a female infant born with a severe form of Goltz syndrome that included an extremely wide facial cleft, an abnormality not previously reported in a patient with this disorder. Her management demonstrates the advantages of a multidisciplinary approach to effectively care for patients with severe craniofacial abnormalities.

scholarly journals Multidisciplinary Treatment Approach in a Patient with History of Nasopharyngeal Carcinoma

2014 ◽  
Vol 2014 ◽  
pp. 1-5
Author(s):  
Atacan Yavuz ◽  
Ömer Birkan Ağralı ◽  
Zeynep Lale Çalışkan ◽  
Dilek Türkaydın ◽  
Atilla Sertgöz ◽  
...  
Keyword(s):  
Nasopharyngeal Carcinoma ◽  
Multidisciplinary Approach ◽  
Dental Treatment ◽  
Treatment Approach ◽  
Multidisciplinary Treatment ◽  
Preventative Care ◽  
Self Confidence ◽  
Dental Hospital ◽  
History Of

Radiotherapy in NPC patients has side effects on the dentition, which affects quality of life dramatically. This case report presents multidisciplinary dental treatment approach in a 17-year-old male patient with a history of nasopharyngeal carcinoma (NPC), which was treated with chemotherapy and radiotherapy. The adolescent patient applied to dental hospital 4 years after the radiotherapy with aesthetic and functional problems on dentition affecting psychological, social, and physical aspects of his life. The dentition of the patient demonstrated the severe destruction as a devastating side effect of radiotherapy. With a successful multidisciplinary approach, our patient’s aesthetics, function, and self-confidence were obtained. Well-established procedures, which include preventative care and maintenance, can reduce the duration and expenses of the treatment and help in challenging the life-long complications of radiotherapy.

scholarly journals A rare case report on double J stenting in a rare case antepartum patient with right sided hydronephrosis and hydroureter

2021 ◽  
Vol 10 (10) ◽  
pp. 3989
Author(s):  
Niranjan N. Chavan ◽  
Shikhanshi . ◽  
Tulika Chouhan ◽  
Shreya Kampoowale
Keyword(s):  
Rare Case ◽  
Multidisciplinary Approach ◽  
Renal Colic ◽  
Symptom Relief ◽  
Severe Form ◽  
Gravid Uterus ◽  
Potential Harm ◽  
Rare Case Report ◽  
Loin Pain ◽  
Surgical Treatments

Gestational hydronephrosis (GH) is caused due to dilatation effect of the progesterone as well as physical pressure of the gravid uterus. In pregnancy, its management is challenging as routine radiological investigations and surgical treatments cannot be performed due to the potential harm to the foetus. Women who fail to respond to conservative methods require intervention. Acute hydronephrosis and renal colic are common aetiologies for loin pain, and can lead to severe form of urinary tract infection affecting perinatal outcome. Double J (DJ) stenting during pregnancy is safe, requiring no intra-operative imaging, and inserted under local anaesthesia. It provides good symptom relief, low complication rate, efficient and safe modality for women with refractory symptoms. Multidisciplinary approach to this procedure is advised.

Alagille syndrome

2011 ◽  
Author(s):  
R. Mark Beattie ◽  
Anil Dhawan ◽  
John W.L. Puntis
Keyword(s):  
Differential Diagnosis ◽  
Clinical Features ◽  
Multidisciplinary Approach ◽  
Alagille Syndrome ◽  
Ocular Involvement ◽  
Multisystem Disorder

Genetics 358Clinical features 358Differential diagnosis 359Investigations 359Management: multidisciplinary approach 360Prognosis 360Alagille syndrome is a complex multisystem disorder with a prevalence of 1:40 000–1:100 000, characterized by hepatic, cardiac, renal, and ocular involvement. At least three of the following five major criteria are required to establish the diagnosis: ...

Multidisciplinary Treatment of Complicated Crown-Root Fractures: A Case Study

2016 ◽  
Vol 41 (6) ◽  
pp. e168-e173 ◽  
Author(s):  
IL Stojanac ◽  
BV Bajkin ◽  
MT Premovic ◽  
BD Ramic ◽  
LM Petrovic
Keyword(s):  
Multidisciplinary Approach ◽  
Multidisciplinary Treatment ◽  
Functional Rehabilitation ◽  
Crown Root ◽  
Reinforced Composite ◽  
Root Fractures ◽  
Traumatic Dental Injuries ◽  
Surgical Conditions ◽  
Dental Injuries

SUMMARY Traumatic dental injuries usually occur among children and adolescents, with maxillary central incisors as the most often affected teeth. Complicated crown-root fractures are particularly challenging for esthetic and functional rehabilitation and often require a multidisciplinary approach. A 21-year-old male patient came to the Dental Clinic due to fractured maxillary incisors caused by trauma during a sporting activity. Clinical examination revealed horizontal fractures of teeth 7, 8, and 9, initiating in the labial cervical third and extending subgingivally on the palate, with exposed pulp tissues. On provisional repositioning and splinting the fragments, root canal treatment was performed. Definitive repositioning was accomplished by raising a full-thickness gingival flap, using fiber-reinforced composite posts, by an endodontist and an oral surgeon. Reattachment was accomplished under surgical conditions to ensure precise positioning of fragments by exposing the palatal aspect of the fracture lines and providing a dry operating field. Definitive composite resin veneers were performed after seven days.

scholarly journals Goldenhar syndrome: a rare entity

2017 ◽  
Vol 4 (5) ◽  
pp. 1897 ◽  
Author(s):  
Tariq Harris ◽  
Mohammed Abdul Bashith ◽  
Manas M. Shanbhag ◽  
Moideen Faheem
Keyword(s):  
Multidisciplinary Approach ◽  
Systemic Treatment ◽  
Obstructive Hydrocephalus ◽  
Severe Form ◽  
Goldenhar Syndrome ◽  
Branchial Arches ◽  
Hydrocephalus Treatment ◽  
Ophthalmic Treatment ◽  
Cns Malformations ◽  
Limbal Dermoid

Goldenhar syndrome was first described by Maurice Goldenhar in 1952 it involves abnormalities in the first and second branchial arches. This syndrome appears to have varying degree of presentation from mild to severe form. Different pathophysiological mechanisms have been proposed .Oculo auriculo vertebral syndrome which includes reduction in blood flow resulting in focal haemorrhage in the first and second branchial arches. In addition there may be cardiac vertebral and CNS defects. A male baby 30-32 wks, preterm SGA was admitted in NICU for evaluation of Congenital Malformations which includes swelling of the anterior border of both eyes with b/l preauricular ear tags, malformed pinna, high arched palate and micrognatia ophthalmology examination revealed b/l limbal dermoid inferotemporal aspect. ECHO done revealed ASD 4mm.CT scan showed obstructive hydrocephalus. Treatment of the condition varies according to the severity. Ophthalmic treatment aimed first because of amblyogenic risk. Craniofacial surgery in view of severe micrognathia systemic treatment may be related to cardiac, renal and CNS malformations. Goldenhar Syndrome is a rare congenital anomaly, with cosmetic defects whose treatment may pose numerous challenges with multidisciplinary approach for optimal management.

scholarly journals A multidisciplinary treatment approach of complete transposition of impacted maxillary left canine with maxillary first premolar in presence of bilateral congenital absence of maxillary lateral incisors

2016 ◽  
Vol 6 ◽  
pp. 39-44
Author(s):  
Pawankumar Dnyandeo Tekale ◽  
Ketan K. Vakil ◽  
Jeegar K. Vakil ◽  
Chetan O. Agarwal
Keyword(s):  
Multidisciplinary Approach ◽  
Tooth Movement ◽  
Multidisciplinary Treatment ◽  
Three Dimensional ◽  
Lateral Incisor ◽  
Current Case ◽  
Restorative Treatment ◽  
Complete Transposition ◽  
Iatrogenic Damage ◽  
Maxillary Lateral Incisor

The maxillary permanent canine is the tooth most frequently involved, which is often transposed with the first premolar followed by transposition between the maxillary permanent canine and maxillary lateral incisor. This study describes the correction of complete transposition of the maxillary left first premolar with impacted permanent maxillary left canine and congenital missing maxillary lateral incisor. The current case report describes a multidisciplinary approach for the alignment of impacted and transposed teeth with surgical exposure of impacted maxillary canine and restorative treatment of transposed maxillary left first premolar. The satisfactory results were achieved with maximum three-dimensional control of tooth movement and without any iatrogenic damage.

scholarly journals A Case of Familial Focal Dermal Hypoplasia: A Report of 3 Cases in Consecutive Generations

2018 ◽  
Vol 2 (5) ◽  
Author(s):  
David Ernesto Castillo ◽  
Nicole Nagrani ◽  
David Castillo ◽  
Rocio Reyes Muñoz ◽  
Mayerlis Cárdenas Guevara ◽  
...  
Keyword(s):  
Skin Lesions ◽  
Dominant Inheritance ◽  
Multisystem Disorder ◽  
Enamel Defects ◽  
Focal Dermal Hypoplasia ◽  
Uncommon Presentation ◽  
Goltz Syndrome ◽  
The Right ◽  
Blaschko’S Lines ◽  
Musculoskeletal Systems

Focal dermal hypoplasia (FDH), or Goltz syndrome, is a rare multisystem disorder affecting mesodermal and ectodermal structures, with the skin, eyes, teeth, and musculoskeletal systems most commonly affected. FDH results from mutations in the PORCN gene. Ninety-five percent of cases arise from novo mutations, whereas 5% are hereditary with an X-linked dominant inheritance pattern. Here, we describe an uncommon presentation of FDH in three consecutive generations. Patient 1 is an 8-month-old female born of non-consanguineous marriage who presented with diffuse alopecia of the scalp, linear hypopigmented, atrophic papules, and plaques with peripheral hyperpigmentation on the left hemiabdomen and right lateral leg along Blaschko's lines as well as syndactyly of the right second and third toes. Skin biopsy from the abdomen showed a thin epidermis with flattened rete ridges and massive dermal edema within collagen fibers and reactive capillaries. Family history was significant for similar skin lesions and bone deformities in her mother and similar skin lesions in her grandmother. Patient 2 (patient 1’s mother) is a 17-year-old female with similar linear hypopigmented, atrophic plaques with peripheral hyperpigmentation on the abdomen and right axilla, syndactyly of the right hand, patchy alopecia of the scalp, microdontia, teeth fusion, enamel defects, verrucous papillomas in the axillae and onycholysis. Patient 3 (patient 1’s grandmother), presented with similar hypopigmented, atrophic plaques on the abdomen and left arm.

scholarly journals Merkel Cell Carcinoma: Interdisciplinary Management of a Rare Disease

2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
Sven Schneider ◽  
Dietmar Thurnher ◽  
Boban M. Erovic
Keyword(s):  
Cell Carcinoma ◽  
Merkel Cell Carcinoma ◽  
Multidisciplinary Approach ◽  
Quality Care ◽  
Contemporary Literature ◽  
Multidisciplinary Treatment ◽  
Interdisciplinary Management ◽  
Merkel Cell ◽  
Tumor Center ◽  
Contemporary Management

Background. The goal of this paper is to review contemporary multidisciplinary treatment with reference to Merkel cell carcinoma. Management of this rare but highly aggressive skin cancer is a complex undertaking that necessitates an understanding of its etiology, epidemiology, clinical presentation, and the coordinated work of several clinical specializations.Recent Findings. The contemporary literature employs a multidisciplinary approach to achieve the best patient's treatment.Conclusion. This paper presents an algorithm for contemporary management for the rare and aggressive Merkel cell carcinoma. Multidisciplinary approach in a tumor center provides high-quality care for patients with Merkel cell carcinoma.

scholarly journals Multidisciplinary treatment of advanced hepatocellular carcinoma with severe arterioportal shunt: a case report

2021 ◽  
Vol 49 (7) ◽  
pp. 030006052110248
Author(s):  
Yao-chang Luo ◽  
Hai-lin Lu ◽  
Wen-ling Song ◽  
Fei-fei Xuan
Keyword(s):  
Hepatocellular Carcinoma ◽  
Multidisciplinary Approach ◽  
Treatment Options ◽  
Multidisciplinary Treatment ◽  
Advanced Hepatocellular Carcinoma ◽  
Term Survival ◽  
Arterioportal Shunt ◽  
Advanced Stages ◽  
Interventional Radiology Procedures

Hepatocellular carcinoma (HCC) is the fourth leading cause of cancer mortality globally. In addition, most patients present in advanced stages with limited curative treatment options. Therefore, multidisciplinary treatment is often warranted. Here, we report a patient with HCC and severe arterioportal shunt (APS) who was treated with a multidisciplinary approach comprising interventional radiology procedures, apatinib and camrelizumab. After treatment, the intrahepatic mass was stable, and a notable decrease in the number and size of lung lesions was observed. The patient achieved a long-term survival of more than 2 years. These data suggest that multidisciplinary treatments may be effective in the treatment of advanced HCC with severe APS.

scholarly journals Oculo-Facio-Cardio-Dental Syndrome: A Case Report about a Rare Pathological Condition

2019 ◽  
Vol 16 (6) ◽  
pp. 928
Author(s):  
José Martinho ◽  
Hugo Ferreira ◽  
Siri Paulo ◽  
Anabela Paula ◽  
Carlos-Miguel Marto ◽  
...  
Keyword(s):  
Orthognathic Surgery ◽  
Orthodontic Treatment ◽  
Multidisciplinary Approach ◽  
Pathological Condition ◽  
Treatment Plan ◽  
Multidisciplinary Treatment ◽  
Clinical Report ◽  
Nasal Bridge ◽  
Septal Defects ◽  
Bilateral Sagittal Split Osteotomy

(1) Background: Oculo-facio-cardio-dental (OFCD) syndrome is a rare pathological condition with an X-linked dominant trait that only occurs in females; no males are born with OFCD syndrome. This syndrome is characterized by congenital cataracts with secondary glaucoma ocular defects, ventricular and atrial septal defects, or mitral valve prolapses. Facial traits are a long narrow face and a high nasal bridge with a bifid nasal tip. Dental anomalies include radiculomegaly, oligodontia, root dilacerations, malocclusion, and delayed eruption. (2) Methods: This clinical report describes a 26-year-old girl who suffers from OFCD syndrome and who was treated with a multidisciplinary approach. The treatment plan included orthodontic treatment, orthognathic surgery, namely LeFort I and a Bilateral Sagittal Split Osteotomy, and occlusal rehabilitation with implants. (3) Discussion: Early diagnosis and multidisciplinary treatment of orthodontic, orthognathic surgery and occlusal rehabilitation with implants make it possible to maintain tooth function and improve aesthetics with good prognoses for success. In this paper, we report a case of a female patient with OFCD syndrome, who was referred for orthodontic treatment and occlusal rehabilitation and treated with a multidisciplinary approach.

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