Cases reported "Achondroplasia"

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1/16. Combined spinal epidural anaesthesia for vesico-vaginal fistula repair in an achondroplastic dwarf.

    A 33-year-old achondroplastic female was scheduled to undergo vesico-vaginal fistula repair by the abdominoperineal route. Preoperative examination suggested a difficult airway so a combined spinal epidural technique was used. Subarachnoid block (sensory loss to T6) was established using 0.5% hyperbaric bupivacaine 1 ml. Anaesthesia was prolonged with incremental doses of epidural bupivacaine 0.5% (total 10 ml) and postoperative analgesia was provided with epidural morphine boluses.
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ranking = 1
keywords = airway
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2/16. Midface distraction to alleviate upper airway obstruction in achondroplastic dwarfs.

    OBJECTIVE: The use of midface distraction in patients with achondroplasia and upper airway obstruction secondary to midface hypoplasia has not been reported. In this report, we review the treatment of two patients with severe midface hypoplasia and obstructive sleep apnea secondary to achondroplasia using midface distraction osteogenesis. DESIGN, SETTING, AND patients: Two patients with achondroplastic dwarfism and midface hypoplasia with airway obstruction were treated in a tertiary referral center for craniofacial disorders. RESULTS AND CONCLUSIONS: Both patients had their tracheostomies decannulated after midface distraction of 25 mm. Midface distraction osteogenesis is useful to alleviate upper airway obstruction from midface hypoplasia seen in achondroplasia.
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ranking = 112.86960460136
keywords = airway obstruction, airway, obstruction
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3/16. An unusual cause of back pain in an achondroplastic man.

    A case is described of a 47-year-old man with achondroplasia who presented with lower back pain radiating to his left loin. An intravenous urogram (IVU) showed hydronephrosis on the left side and a dilated left ureter passing down into the left inguinal region. A CT scan confirmed a left inguinal hernia containing the left ureter causing ureteric obstruction. The hernia was repaired using the Lichtenstein technique with the ureter replaced retroperitoneally. A postoperative IVU showed recovery in renal function but with a persistently dilated left ureter that was not obstructed. A review of the literature regarding inguinal herniation involving the ureter is presented.
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ranking = 0.37899074464298
keywords = obstruction
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4/16. Anaesthetic management of a patient with achondroplasia.

    A 12-year-old girl diagnosed with achondroplasia was admitted for bilateral ear surgery and adenotonsillectomy. She had classical symptoms and signs of upper airway obstruction, which is often seen in patients with achondroplasia. We describe the anaesthetic management of this patient, emphasizing the airway difficulties encountered and their anaesthetic implications.
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ranking = 17.124229228765
keywords = airway obstruction, airway, obstruction
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5/16. hydrocephalus in an achondroplastic child treated by venous decompression at the jugular foramen. Case report.

    A 10-month-old child with achondroplasia with progressive head enlargement, ventriculomegaly, and wide subarachnoid spaces over the hemispheres was referred for evaluation. A steady-state lumbar infusion test revealed increased cerebrospinal fluid (CSF) outflow resistance (14 mm Hg/ml/min), and intra-arterial digital subtraction angiography (DSA) demonstrated bilateral venous outflow obstruction due to stenosis of the jugular foramen. Surgical decompression by opening the right jugular foramen relieved the clinical signs of intracranial hypertension. During the following year, the patient's head enlargement was moderate with relative normalization of size. Repeat DSA demonstrated improved venous runoff on the right side, and a steady-state lumbar infusion test demonstrated reduced CSF outflow resistance (10 mm Hg/ml/min). Venous decompression is causal therapy and may prove to be preferable to shunting in children with hydrocephalus and bilateral stenosis of the jugular foramen.
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ranking = 0.37899074464298
keywords = obstruction
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6/16. hydrocephalus in achondroplasia: the possible role of intracranial venous hypertension.

    The significance and cause of ventriculomegaly in achondroplasia was investigated in five achondroplastic children. The intraventricular pressure (IVP) was monitored over 24 hours, followed by intraventricular injection of radionuclide alone or in combination with water-soluble contrast material. The IVP was elevated and the reabsorption of cerebrospinal fluid (CSF) into the sagittal sinus was slow in all cases, but there was no obstruction to CSF flow. The spinal subarachnoid space was well seen in all patients. Jugular venograms with pressure monitoring were obtained in four patients (bilaterally in one). These studies confirmed a narrow jugular foramen in all patients with a significant venous pressure gradient (3 to 10 mm Hg) obtained while the catheter was being pulled back from the sigmoid sinus through the foramen. A second gradient was found in the jugular vein in two patients at the level of the upper thoracic aperture. This gradient was 6 and 14 mm Hg, respectively. Identical venograms and monitoring of the venous pressure in a control group showed no pressure gradients across the jugular foramen and smaller gradients (2 to 5 mm Hg) across the thoracic inlet. It is concluded from these studies that ventriculomegaly in achondroplastic children represents hydrocephalus, which is likely secondary to raised intracranial venous pressure due to hemodynamically significant stenosis of the jugular foramen and, in some cases, the jugular vein in the thoracic aperture.
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ranking = 0.37899074464298
keywords = obstruction
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7/16. foramen magnum decompression in an infant with homozygous achondroplasia. Case report.

    Homozygous achondroplasia is a rare yet distinct clinical entity. Most infants succumb to an early death as a result of respiratory compromise due to upper airway obstruction, thoracic cage deformity, and/or cervicomedullary compression. The successful cervicomedullary decompression of a 16-week-old infant with homozygous achondroplasia is described. This report suggests that homozygous achondroplasia is not universally fatal and that these infants are potentially viable if managed by aggressive respiratory and surgical measures.
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ranking = 16.124229228765
keywords = airway obstruction, airway, obstruction
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8/16. Reversibility of deficient sleep entrained growth hormone secretion in a boy with achondroplasia and obstructive sleep apnea.

    Obstructive sleep apnea may lead to disordered sleep architecture and impair the physiologic slow wave sleep related growth hormone release. Obstructive sleep apnea occurs with craniofacial syndromes and in children with airway narrowing, pharyngeal hypoplasia, tonsillar adenoidal hypertrophy, micrognathia and achondroplasia. To examine the relationship between disordered sleep and growth hormone release we studied a 9 year old male with achondroplasia, growth failure (3 cm/year) and obstructive sleep apnea. polysomnography data and a 20 min sampling for sleep entrained growth hormone showed before therapeutic tracheostomy numerous apneic episodes, absent slow wave sleep and abnormal low growth hormone secretion during sleep. Normalized slow wave sleep entrained growth hormone secretion after tracheostomy led to a sustained increase in growth rate. Normal growth rate (greater than 5 cm/year) continues 2 years after tracheostomy. We conclude that obstructive sleep apnea may impair sleep related growth hormone release. Obstructive sleep apnea may be a useful model for other diseases in which growth failure and sleep disturbances are linked.
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ranking = 1
keywords = airway
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9/16. Postoperative macroglossia causing airway obstruction.

    macroglossia can cause a wide spectrum of problems in the pediatric population. airway obstruction is the most severe of the sequelae and must be handled promptly. Typically, congenital macroglossia does not present with sudden airway obstruction, however, traumatic or postoperative macroglossia can. Iatrogenic postoperative macroglossia is presented and mechanisms of injury discussed. Preventative and therapeutic measures are outlined.
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ranking = 66.254898404347
keywords = airway obstruction, airway, obstruction
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10/16. Partial upper airway obstruction and sleep apnoea.

    An unusual case is described in which partial upper airway obstruction led to a sequence of events characterized by sleep disturbance, enuresis, somnolence and cardio-respiratory collapse. The upper airway obstruction was not immediately apparent as the cause of subsequent clinical events. attention is drawn to the general medical problems which may be produced by upper airway obstruction, with particular reference to those accompanying obesity and abnormal stature.
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ranking = 112.86960460136
keywords = airway obstruction, airway, obstruction
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