Soft Tissue Hypertrophy Research Articles

Complications of bone-anchored hearing aids in pediatric patients

Objective To describe the range and rate of complications related to bone-anchored hearing aids in pediatric patients. Method We conducted a retrospective review all children 16 years of age or less who were implanted with a bone-anchored hearing aid from 2002 to 2009. The main outcome measures were post Baha ® implantation complications including infection, soft tissue hypertrophy, loss of osseointegration, and need for further surgery. Results Thirty-one patients were identified and 27 had sufficient follow up for complete analysis. Soft tissue reactions were seen in 24 patients (89%), half of which were considered minor. Ten patients (37%) had major complications defined as requiring revision surgery or removal of the implant. Soft tissue overgrowth and infection at the abutment required removal in 5 children (19%) and three implants (11%) failed to osseointegrate. Recurrent antibiotic treatment was required in eight patients (30%). Seven patients (26%) had a history of trauma and one of them lost the implant. To date, three of the children (11%) have been unable to use the device because of chronic infection and overgrowth of the abutment. There was a statistically significant increased risk for major complications in patients from socioeconomically deprived backgrounds and obesity appears to incur additional risk. Conclusions Complications of bone-anchored hearing aid implants are common in our experience and obesity and socioeconomic factors appear to contribute to a higher risk for complications. Frequent follow up and meticulous care of the implant site may minimize complications but can be challenging in this population.

Anesthesia for Surgery Related to Klippel–Trenaunay Syndrome

Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation characterized by the triad of varicose veins or venous malformations, capillary malformations that may involve neurovascular structures, and bony or soft tissue hypertrophy in affected limbs. Areas such as the trunk, bowel, bladder, and spinal cord may be involved as well. KTS should not be confused with Klippel-Feil syndrome, which involves abnormalities of the cervical vertebrae. Anesthetic management for patients with KTS has only been described in limited case reports that caution about potential airway difficulty but do not report surgical hemorrhage requiring transfusion. We performed an electronic search of the Mayo Clinic medical record database to identify patients who had undergone an anesthetic for surgery related to KTS. Review of medical records was performed for type of surgery, anesthetic technique, airway management and difficulty, medications used, intraoperative fluid administration, transfusion requirements, vascular access used, and postoperative complications. Eighty-two unique patients were identified who underwent 134 general anesthetics and 2 lumbar neuraxial anesthetics for surgeries related to KTS. Preoperatively, 27% of patients had a history of recurrent bleeding, 24% recurrent cellulitis, 9% deep vein thrombosis, and 2% pulmonary embolism. The mean age at time of surgery was 21 ± 15 years. The majority of surgical procedures involved laser coagulation or varicose vein sclerotherapy or stripping. All of the 74 direct laryngoscopies and tracheal intubations were performed on the first attempt without difficulty. Mask ventilation was possible in all 131 patients for whom this was attempted, with only 1 requiring an oral airway. Documented estimated blood loss ranged from 20 to 18,000 mL, with a mean of 740 ± 2739 mL. Use of a tourniquet did not obviate the possibility of substantial blood loss. The only significant postoperative complication involved a calf hematoma after vein stripping and avulsion that required return to the operating room for evacuation. Patients with KTS have multiple associated comorbidities relevant to perioperative management. In contrast to previous reports, difficulty with airway management was not encountered. Surgery related to severe KTS may be associated with massive hemorrhage despite tourniquet use, and the anesthesiologist should anticipate the need for appropriate fluid resuscitation. Neuraxial techniques may be considered only if the possibility of trauma to neurovascular malformations has been excluded with recent spine imaging.

Rhinophyma: Excision and Reconstruction with Local Nasolabial Flap

ABSTRACT Rhinophyma is a rare disease which causes disfiguring soft tissue hypertrophy of the nose. Various medical and surgical treatment modalities have been proposed and tried with varying degree of success. We report a case where we adopted excision of the rhinophymatous tissue and reconstructed with the local nasolabial flap. The result was cosmetically good enough and the procedure technically simple.

Pathophysiology and Surgical Strategies in the Management of the Ossified Spinal Ligaments

Acromegaly is a relatively rare neuroendocrine disorder associated with diffuse hypertrophy of bony and soft tissues due to growth hormone hypersecretion from a pituitary adenoma. Acromegaly can also cause numerous pathological changes in the spine, including degenerative osteoarticular disease, axial arthropathy, spinal stenosis, vertebral fracture and diffuse idiopathic skeletal hyperostosis (Forestier’s disease). Ossified ligamentum flavum (OLF) is a rare disorder that often presents as thoracic spinal stenosis, but to our knowledge has never been described in patients with acromegaly. Previously, no link has been established between these two entities. We present, to our knowledge, the first reported case of OLF in a patient with acromegaly who presented with thoracic spinal cord compression. OLF is a potential spinal manifestation of acromegaly and should be considered in the differential diagnosis of spinal stenosis or spinal cord compression in the context of growth hormone hypersecretion.

Virtual Humans Versus Standardized Patients: Which Lead Residents to More Correct Diagnoses?

Medical educators frequently use standardized patient (SP) encounters to bridge the gap between didactic education and practical application. Typically, SPs are healthy adults with no consistent physical findings; however, highly immersive virtual humans (VHs) may enable the consistent presentation of abnormal physical findings to multiple learners across multiple repetitions. Thus, the authors conducted this study to compare how frequently junior anesthesiology residents suspected obstructive sleep apnea (OSA) in preoperative assessments of SPs versus a VH. The authors presented a patient whose case included the historical features of OSA (snoring, daytime fatigue, observed apnea, hypertension, and obesity). Three SPs (in 2008) and one VH (in 2009) were necessary to run the residents through the assessment. The VH appeared morbidly obese and had a neck circumference of 40 cm [corrected]. An airway exam of the VH displayed an image of redundant soft tissue, prominent tongue, and tonsillar hypertrophy. The VH responded to natural speech by recognizing "triggers" in a human's voice. The 849 triggers and 259 VH responses were designed with a technique that collects information from user interactions. Five of 21 residents (23.8%) suspected OSA after interviewing the SPs, whereas 11 of 13 residents (84.6%) suspected OSA after interviewing the VH (odds ratio of 17.6; 95% CI of 2.9-107). Residents suspected OSA much more frequently after interviewing the VH than after interviewing the SPs. The VH provides a unique opportunity to display numerous abnormal physical findings as part of SP encounters.

Bilateral optic nerve drusen and gliomas in Klippel-Trenaunay syndrome

Klippel-Trenaunay syndrome (KTS) consists of a vascular nevus involving an extremity, varicosities of that extremity, and hypertrophy of bone and soft tissue. When arteriovenous malformation is also present, it is called Klippel-Trenaunay-Weber syndrome (KTWS). Ophthalmic features of these syndromes include vascular anomalies of the orbit, iris, retina, choroid, and optic nerve. We report a case of a 16-year-old girl with KTS who was found to have bilateral optic nerve and chiasmal gliomas, optic disk drusen, and acquired myelination of the retinal nerve fiber layer. These findings have not been previously reported to be associated with KTS or KTWS.

Thoracic vertebral hemangioma causing paraplegia in klippel-trenaunay-weber syndrome: case report

Vertebral hemangiomas are the most common tumours of the vertebral column. Generally, these tumours are asymptomatic but some patients complain of back pain and develop neurologic symptoms due to extraosseous extension. Vertebral hemangiomas can extend extradurally causing neurological impairment as a result of compression of the spinal cord and nerve roots. Vertebral hemangiomas may be multiple and detectable as a component of the Klippel-Trenaunay-Weber syndrome. Although this syndrome consists of deep venous thrombosis, lymphatic anomalies, cutaneous capillary malformations, and hypertrophy of soft tissue and bone on extremities, its clinical presentation may be very variable. We present a unique case of vertebral hemangioma causing spinal cord compression due to the extradural extension that also had deep venous thrombosis, hematuria, hypophyseal cyst and ventricle asymmetry, diagnosed as the Klippel-Trenaunay-Weber syndrome.

Overlap of Sturge-Weber syndrome and Klippel-Trenaunay syndrome

Sturge–Weber syndrome is a rare sporadic condition of mesodermal phakomatosis, characterized by purple-colored flat cutaneous cranial (face) hemangiomas (most commonly along the trigeminal nerve), glaucoma and vascular lesions in the ipsilateral brain and meninges. Klippel–Trenaunay syndrome is also an uncommon mesodermal phakomatosis characterized by a triad of cutaneous and visceral hemangiomas, venous varicosities and soft tissue or bone hypertrophy. Sturge–Weber syndrome in combination with Klippel–Trenaunay syndrome is unusual. Because of the rarity, we report here a 3-year-old boy who presented with overlapping features of both the syndromes.

Involvement of Splenic Hemangioma and Rectal Varices in a Patient with Klippel - Trenaunay Syndrome

Klippel - Trenaunay syndrome (KTS) is characterized by a cutaneous vascular nevus of the involved extremity, bone and soft tissue hypertrophy of the extremity and venous malformations. We present a case of KTS with splenic hemangiomas and rectal varices. A 29-year-old woman was referred for intermittent hematochezia for several years. She had history with a number of operations for cutaneous and soft tissue hamangiomas since the age of one year old and for increased circumference of her left thigh during the last few months. Abdominal CT revealed multiple hemangiomas in the spleen, fusiform aneurysmal dilatation of the deep veins and soft tissue hemangiomas. There was no evidence of hepatosplenomegaly or liver cirrhosis. Colonoscopy revealed hemangiomatous involvement in the rectum. There were rectal varices without evidence of active bleeding. Upon venography of the left leg, we also found infiltrative dilated superficial veins in the subcutaneous tissue and aneurysmal dilatation of the deep veins. The patient was finally diagnosed with KTS, and treated with oral iron supplementation only, which has been tolerable to date. Intervention or surgery is not required. When gastrointestinal varices or hemangiomatous mucosal changes are detected in a young patient without definite underlying cause, KTS should be considered.

Assisted reproduction in a patient with Klippel-Trenaunay syndrome: management of thrombophilia and consumptive coagulopathy

Klippel-Trenaunay Syndrome (KTS) is a rare, sporadic triad of congenital malformations involving an extensive port wine stain, soft tissue or bone hypertrophy and underlying venous and/or lymphatic malformation involving an extremity. Pregnancy is known to exacerbate KTS complications and can put women at increased obstetrical risk due to deep venous thrombosis and other thromboembolic events. Here we report a case of a patient with KTS who achieved a pregnancy through in vitro fertilization (IVF) using her own eggs and a gestational surrogate in the setting of hypercoagulability and chronic consumptive coagulopathy.

“Impingement” anteriore di caviglia nello sportivo

Anterior ankle impingement is a syndrome characterized by pain during movement, mainly dorsiflexion, and sometimes by functional restriction. This syndrome predominantly affects athletes, but could be found also in people with lower functional demands with an history of previous untreated or poorly treated ankle sprain. In this clinical feature we can find the following joint lesions: soft tissue hypertrophy, osteocartilagineous tissue damage, osteophytic formations. History and physical examination are very useful to reach an early diagnosis, which is recommended to prevent progression to a more compromised situation. In those cases in which conservative treatment did not solve the problem we consider ankle arthroscopy an excellent diagnostic tool in order to find specific lesions which have been undetected in previous instrumental investigations; at the same time it enables to perform surgery bringing the benefits of minimal invasiveness, low post-surgical rate complications and early recovery to rehabilitation treatment.

P09.07: Prenatal diagnosis of Klippel Trenaunay syndrome: a case report

Klippel Trenaunay syndrome is a rare congenital disease that is characterized by a triad of cutaneous capillary malformations, varicose veins and hypertrophy of bone and soft tissue, usually asymmetric in its presentation (a single extremity). This disease tends to be sporadic, with very few familiar occurrences and postulated to be related to a somatic mutation for a critical angiogenetic-vasculogenetic factor during embryonic development. The prenatal diagnosis of this syndrome has been scantly referred. We present the case of a 29 year old pregnant woman, with two previous normal pregnancies and babies, who came to our unit for a routine morphologic scan at 18.4 weeks. First trimester scan had not been performed. The fetus was structurally normal, with a choroid plexus cyst and single umbilical artery. The most striking findings were the presence of cystic and heterogenous images affecting both legs, from the gluteus to below the knee, with predominance of the left one. A significant asymmetry between both legs, specially in thickness, was seen. An additional cystic image was found below the left kidney. Angiopower color Doppler demonstrated increased vascularisation of the mass. Karyotype by amniocentesis shown to be normal 46XX. In view of all of these findings, with asymmetric limb hypertrophy and the presence of multiple hemangiomas, and arteriovenous fistulas, a very likely diagnosis of Klippel Trenaunay syndrome was suggested to the parents. After extensive counselling, parents opted for termination of pregnancy at 20.4 weeks. Pathological examination results confirmed the diagnosis.

PROMINENT OROFACIAL FINDINGS IN KLIPPEL-TRENAUNAY SYNDROME

Klippel-Trenaunay syndrome is a triad of congenital anomalies characterized by nevus flammeus, varicosities, and unilateral bony and soft tissue hypertrophy. Orofacial manifestations include facial asymmetry, jaw enlargement, and malocclusions as well as premature tooth eruption. Presented here is an illustrative report of Klippel Trenaunay syndrome in a 30 year old male patient showing all the characteristic findings.

E-015 Onyx embolization of facial arteriovenous malformations

IntroductionVascular malformations of the head and neck encompass a wide range of lesions, including lymphatic malformations, hemangiomas, venous malformations and true arteriovenous malformations. Treatment of these lesions is challenging because...

Multiple cerebral and spinal cord cavernomas in Klippel-Trenaunay-Weber syndrome

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare syndrome in which patients harbor cutaneous hemangiomas, venous varicosities, and osseous–soft tissue hypertrophy of the affected limb. The clinical presentation of this syndrome is variable and the etiopathogenesis is presumably genetic in view of recent discoveries of RASA1 gene mutations in KTWS patients. Similarly, the KRIT1 gene is involved in pathogenesis of cavernous angiomas. Both RASA1 and KRIT1 genes interact with Rap1a protein, a member of the Ras family of guanosine triphosphatases (GTPases) signalling cellular adhesion. We report a 55-year-old male with KTWS harboring multiple cavernous angiomas in the thoracic spinal cord and the brainstem, as revealed by MRI. Angiography ruled out arteriovenous malformation. The patient was managed conservatively. The rarity of cavernous angiomas in KTWS and the possibilities of shared genetic pathways between KTWS and cavernous angiomas are discussed.

Pulmonary thromboendarterectomy in Klippel–Trénaunay syndrome

Chronic thromboembolic pulmonary hypertension (CTEPH) occurs in 4% of patients with pulmonary embolism (PE).1 Untreated, the likelihood of survival is poor. Klippel–Trénaunay syndrome (KTS) is a rare complex vascular malformation that typically includes the triad of capillary vascular malformation (port-wine stain), venous malformation(s), and soft tissue or bony hypertrophy.2 Patients with KTS are at increased risk for peripheral venous thrombosis and pulmonary emboli.3 When medical management of PE and CTEPH fails, pulmonary thromboendarterectomy (PTE) is an important, potentially lifesaving therapy.

Persistent fetal vasculature and spontaneous hyphema in a patient with Klippel-Trénaunay-Weber syndrome

Klippel-Trénaunay-Weber syndrome is characterized by the triad of capillary malformations (usually port-wine stains), varicose veins or venous malformations of unusual distribution, and soft-tissue or bony hypertrophy of an extremity. The syndrome can be diagnosed on the basis of 2 of these 3 features. In the atypical form of the syndrome, capillary malformations may be absent. Recognition is possible during infancy or early childhood, and evaluation and treatment is important to minimize morbidity. We describe the case of an 11-year-old girl who presented with hyphema after a Valsalva maneuver and was found to have persistent fetal vasculature in the affected eye. She had systemic features of Klippel-Trénaunay-Weber syndrome. Magnetic resonance imaging, magnetic resonance angiography, and magnetic resonance venography scans of the brain and orbits were normal. Magnetic resonance imaging scan of the left hypertrophied lower limb revealed venous malformations leading to soft-tissue hypertrophy. To our knowledge, this is the first case of persistent fetal vasculature and hyphema in a patient with Klippel-Trénaunay-Weber syndrome.

Syndrome de Klippel-Trenaunay

Klippel-Trenaunay syndrome (KTS) is a rare complex vascular congenital malformation. The characteristic triad is an association of a cutaneous capillary angioma of a limb, venous malformations, and hypertrophy of soft tissue and/or bone. Diagnosis is essentially clinical. Work-up of the lesion may involve noninvasive imaging: Doppler ultrasound, standard radiography, or magnetic resonance imaging (MRI). The presence of arteriovenous malformations is sought by clinical examination or ultrasound: they rule out a diagnosis of KTS. Management is multidisciplinary and involves especially venous control and orthopedic management of unequal limb lengths.

Síndrome de Klippel-Trenaunay: relato de caso

Klippel-Trenaunay syndrome is characterized by a triad of port-wine stain, varicose veins with or without venous malformations, and bony and soft tissue hypertrophy. It usually affects only one extremity. Its cause continues to be investigated, although theories abound. There is no predilection for gender or any particular ethnicity, and it appears more frequently at birth, childhood or adolescence. We report the case of an 8-year-old boy with this syndrome.

Klippel-Trenaunay Syndrome: a case report with brief review of literature

Klippel-Tranuanay Syndrome is a rare sporadic disease characterized by clinical triad of capillary malformation; soft tissue and bony hypertrophy; and atypical varicosity. Although this syndrome was first described more than hundred years ago, exact incidence has not been estimated yet. Several theories have been postulated to describe its pathogenesis. Clinical presentation of this syndrome is protean ranging from minimal asymptomatic disease to life threatening bleeding and embolism. Management of this syndrome includes careful diagnosis, prevention and treatment of complications. We are presenting a case of Klippel-Tranuanay Syndrome in a 19-year-old male patient presenting with varicosity in lateral aspect of right leg. On careful examination other components of the syndrome were found. Patient was kept on yearly follow up. Usually patients with Klippel-Tranuanay Syndrome present with complication. This case shows that there is also a need for medical solutions in patients who seek advise for cosmetic reasons.