Arthrogryposis-renal dysfunction-cholestasis syndrome is a rare lethal disorder that involves multipl organ system. Three cardinal findings of this syndrome are arthrogryposis, renal tubular dysfunction and cholestasis. The other organ involvements including ichthyosis, central nervous system malformation, platelet anomalies, congenital heart defects and severe failure to thrive are sometimes associated with this syndrome. Clinical findings, organ biopsy and mutational analysis can help for diagnosing but there is no curative treatment except supportive care. Several symptoms of this condition are already usually present in the neonatal period: arthrogryposis, neonatal cholestasis, skin lesions, among others.

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La Habana, Cuba. Con el uso de sutura polipropileno 4. Objective: to evaluate the surgical outcomes of the Crawford technique through the use of silastic and polypropylene 4.

Those patients with prior surgery to treat this disease were excluded. Using polypropylene sutures 4. Conclusions: ptosis was more frequent in male children younger than 8 years. Simple congenital ptosis predominated, either unilateral or severe. Crawford technique was effective in most cases and with similar results using both materials.

Key words: congenital eyelid ptosis, frontalis suspension, blepharoptosis. Presenta epicanto inverso, ptosis y hendidura palpebral transversal disminuida. Mediante el uso de asuppro 4. Cir Pediatr. La enciclopedia libre. Ptosis [Internet]. Ptosis palpebral infantil. En: Fonseca Santodomingo A. Ophthal Plast Reconstr Surg. Rev Mex Oftalmol. Studium [Internet]. Comparison of silastic and banked fascia lata in pediatric frontalis suspension.

J Pediatr Ophthalmol Strabismus. Kashkouli MB. A randomized clinical trial of two methods of fascia lata suspension in congenital ptosis. Long-term results of autogenous palmaris longus frontalis sling in children with congenital ptosis. Results of levator excision followed by fascia lata brow suspension in patients with congenital and jaw-winking ptosis. Correction of congenital severe ptosis by suspension of a frontal muscle flap overlapped with an inferiorly based orbital septum flap.

Aesthetic Plast Surg. Matsuo K, Yuzuriha S. Frontalis suspension with fascia lata for severe congenital blepharoptosis using enhanced involuntary reflex contraction of the frontalis muscle.

J Plast Reconstr Aesthet Surg. Broad fascia fixation enhances frontalis suspension. J Pediatr Ophthalmol. Frontalis suspension for upper eyelid ptosis: evaluation of different surgical designs and suture material. Am J Ophthalmol. Frontalis sling operation using silicone rods in comparison to ptose-up for congenital ptosis with poor levator function. Correction of severe ptosis with a silicone implant suspensor: 22 years of experience.

Plastic Reconstr Surg. Recibido: 19 de junio de Aprobado: 18 de octubre de Servicios Personalizados Revista.

Citado por SciELO. Similares en SciELO.


Tratamiento y desenlace obstétrico de una paciente con artrogriposis múltiple congénita.

Important User Information: Remote access to EBSCO's databases is permitted to patrons of subscribing institutions accessing from remote locations for personal, non-commercial use. However, remote access to EBSCO's databases from non-subscribing institutions is not allowed if the purpose of the use is for commercial gain through cost reduction or avoidance for a non-subscribing institution. Source: Ginecologia y Obstetricia de Mexico. Pregnant patients with this condition have a higher risk of complications in obstetric management. Received advice on the cardiovascular and respiratory system risks, secondary to the underlying disease, that could be exacerbated by pregnancy. The ultrasound during the 22nd week of pregnancy did not report any structural alterations. The patient continued in prenatal control, without respiratory difficulty or cardiovascular alterations.


2017, Número 4

We report a new sporadic case of Freeman-Sheldon syndrome. The parents were not blood relatives. In addition, the patient showed other clinical manifestations such as a large bilateral inguinal hernia and thoracic cage abnormalities. The latter abnormality led to serious episodes of bronchopneumonia that delayed the surgical repair of bilateral inguinal hernia. Knowledge of the sonographic characteristics of deformities of the extremities is essential to reach an early prenatal suspected diagnosis of Sheldon-Freeman syndrome, especially in families with a history of the syndrome.


2011, Número 1

Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients. Eur Respir J ; Rev Esp Anestesiol Reanim ; Rudas F.


La artrogriposis nuevo protócolo de tratamiento


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