Genetically transmitted syndrome characterized by the
association of eye, ear, teeth, and hand abnormalities. The potential association of cardiac defects
cannot be excluded.
Levy Hollister Syndrome; Limb Malformations-Dento-Digital
Exact incidence unknown. Only 12 cases reported in the
Autosomal dominant inheritance with variable
Suspected disorder of mesomelic development.
Wide variability in clinical expression.
Persistent dry mouth because of decreased salivation caused by aplasia or
hypoplasia of the salivary glands. Other features include severe dental caries; nasolacrimal duct
obstruction with chronic epiphora and dacryocystitis; absent lacrimal gland;
peg-shaped teeth with enamel hypoplasia; cup-shaped ear(s) and sometimes
hearing loss; preaxial digital anomalies (triphalangeal thumb,
digitalization of thumb, syndactyly, radial hypoplasia) and clinodactyly of
the fifth digit. Other features less commonly associated include congenital
renal disease (bilateral renal agenesis, cystic dysplastic kidneys) and
dysmorphic facies with retrognathia, high forehead, and deep metopic
fissure. One patient was reported to have a complex cardiopulmonary
malformation: right diaphragmatic palsy (which was present in asymptomatic
form in the mother also), hypoplasia of the left lung vasculature without
bronchial anomalies, mild hypoplasia of the left pulmonary artery, and, on
echocardiography, aneurysm of the interventricular septum.
The potential for difficult direct
laryngoscopy and tracheal intubation must be evaluated carefully clinically
and radiologically if needed. A thorough examination of the renal,
pulmonary, and cardiac systems must be performed, although the association
of anomalies is not frequent but significant when part of the clinical
picture. Pulmonary hypertension may be present because of pulmonary vascular
hypoplasia and should be specifically investigated (e.g., arterial blood
gases, chest radiographs, ultrasound, CT scan, and echocardiography). The
laboratory investigation should include electrolytes, creatinine, blood urea
nitrogen (BUN), and coagulation profile.
The possibility of difficult
laryngoscopy and tracheal intubation is present. Brittle teeth may damage
easily; therefore dentition must be checked prior to oral instrumentation. The
presence of feet and hands abnormalities may make placement of intravenous
and arterial catheters difficult. Diaphragmatic palsy may increase
postoperative respiratory complications. Pulmonary hypertension may be
present because of pulmonary vascular hypoplasia, and the anesthetic
management should be adapted according to this clinical presentation. The
fluid regimen should be adapted to the renal function.
Preservation of spontaneous
ventilation until confirmation that either face-mask ventilation or tracheal
intubation can be achieved easily must be ensured before neuromuscular
blocking agents are used. Avoid atropine if possible because of the presence
of hypoplasia of the salivary glands and potential consequences of severely drying
Azar T, Scott JA, Arnold JE, Robin NH: Epiglottic hypoplasia associated
with lacrimo-auriculo-dental-digital syndrome. Ann Otol Rhinol Laryngol
Francannet C, Vanlieferinghen P, Dechelotte P, et al: LADD syndrome in five
members of a three-generation family and prenatal diagnosis. Genet Counsel
Heinz GW, Bateman JB, Barrett DJ, et al: Ocular manifestations of ...