Kleeblattschadel Anomaly

The word Kleeblattschadel is German for cloverleaf and describes the most severe form of craniosynostosis. It consists of a trilobular-shaped skull (Fig. 26.6), with synostosis of multiple sutures, which most commonly involves the cranial base, facial and cranial vault sutures, with the sagittal, squamosal and metopic remaining open, leading to compensatory bulging through them. The degree of severity varies with the involvement of the different sutures. There can also be a complete cranial vault synostosis (pansynostosis). The etiology is heterogeneous in that it may present as an isolated anomaly or together with other anomalies, making up various syndromes. Approximately 40% of all cases represent thanatophoric dysplasia and approximately 20% represent Pfeiffer syndrome [23].

Craniofacial features include recessed supraorbital rims, frontal bone hypoplasia, maxillary hypoplasia, severe exorbitism, hypertelorism, down-slanting palpebral fissures and also convexity of the squamous portion of the temporal bones. The ears are displaced downward, the nasal bridge is low and the eyelids often fail to close, leading to corneal ulceration and venous distention of the scleras. Radiographically, the skull is trilobular, with a honeycomb appearance and a thin distorted vault.

Hydrocephalus is common, as well as psy-chomotor retardation, cerebellar hypoplasia and herniation, along with other brain anomalies [24]. Other malformations may include blindness, obstructed nasolacrimal ducts, facial clefting, cleft lip/palate, absent external auditory canals, PDA and ASD. Early death has been noted in most cases, with few surviving into adolescence, often due to airway abnormalities that may be present at multiple levels.

Fig. 26.5. a Carpenter syndrome with lambdoidal craniosynostosis, low-set ears, short neck, obesity, down-slanting palpebral fissures and epicanthal folds. b Carpenter syndrome, with brachydactyly of the phalanges and partial soft tissue syndactyly. c Carpenter syndrome, with polysyndactyly of the feet.

Fig. 26.5. a Carpenter syndrome with lambdoidal craniosynostosis, low-set ears, short neck, obesity, down-slanting palpebral fissures and epicanthal folds. b Carpenter syndrome, with brachydactyly of the phalanges and partial soft tissue syndactyly. c Carpenter syndrome, with polysyndactyly of the feet.

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