Semi-Automatic Volumetric Segmentation of the Upper Airways in Patients with Pierre Robin Sequence

Pierre Robin malformation is a rare craniofacial dysmorphism whose pathogenesis is multifactorial. Although there is some agreement in non-invasive treatment in less severe cases, the dispute is still open on cases with severe respiratory impairment. We present a semi-automatic novel diagnostic tool for calculating upper airway volume, in order to eventually address surgery in patients with Pierre Robin Sequence (PRS). Multidetector CT datasets of two patients and two controls were tested to assess the proposed method for ROI segmentation, upper airway volume computation and three-dimensional reconstructions. The experimental results show an irregular pattern and a severely reduced cross-sectional area (CSA) with a mean value of 8.3808 mm2 in patients with PRS and a mean CSA value of 33.7692 mm2 in controls (a ΔCSA of about −75%). Moreover, the similarity indexes and sensitivity/specificity values obtained showed a good segmentation performance. In particular, mean values of Jaccard and Dice similarity indexes were 91.69% and 94.07%, respectively, while the mean values of specificity and sensitivity were 96.69% and 98.03%, respectively. The proposed tool represents an easy way to perform a quantitative analysis of airway volume and useful 3D reconstructions.

[1]  D. Mackay Controversies in the Diagnosis and Management of the Robin Sequence , 2011, The Journal of craniofacial surgery.

[2]  J. Marsh,et al.  Mandibular Distraction Osteogenesis for Pierre Robin Sequence: What Percentage of Neonates Need It? , 2008, The Journal of craniofacial surgery.

[3]  J. Mulliken,et al.  Robin sequence: a retrospective review of 115 patients. , 2006, International journal of pediatric otorhinolaryngology.

[4]  C. Bijnen,et al.  Tongue-Lip Adhesion in the Treatment of Pierre Robin Sequence , 2009, The Journal of craniofacial surgery.

[5]  J. Sidman,et al.  Resolving Feeding Difficulties With Early Airway Intervention in Pierre Robin Sequence , 2008, The Laryngoscope.

[6]  J. Sidman,et al.  Pierre Robin Sequence: evaluation, management, indications for surgery, and pitfalls. , 2012, Otolaryngologic clinics of North America.

[7]  W. D. Pielou Non-surgical management of Pierre Robin syndrome. , 1967, Archives of disease in childhood.

[8]  P. Jaccard Distribution de la flore alpine dans le bassin des Dranses et dans quelques régions voisines , 1901 .

[9]  R. Schreiner,et al.  Stickler syndrome in a pedigree of Pierre Robin syndrome. , 1973, American journal of diseases of children.

[10]  L. Caouette‐Laberge,et al.  Subperiosteal release of the floor of the mouth in Pierre Robin sequence: experience with 12 cases. , 1996, The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association.

[11]  L. R. Dice Measures of the Amount of Ecologic Association Between Species , 1945 .

[12]  Nasopharyngeal airways in Pierre Robin syndrome , 1982 .

[13]  L. Birnbaum,et al.  Hereditary factors in the Pierre Robin syndrome. , 1971, British journal of plastic surgery.

[14]  N. Tommerup,et al.  The Genetic Basis of the Pierre Robin Sequence , 2006, The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association.

[15]  N. Otsu A threshold selection method from gray level histograms , 1979 .

[16]  F. Denoyelle,et al.  Physiologic and Clinical Benefits of Noninvasive Ventilation in Infants With Pierre Robin Sequence , 2010, Pediatrics.

[17]  J. Bosma,et al.  Prone feeding of infants with the Pierre Robin syndrome. , 1966, The Cleft palate journal.

[18]  M. Cohen Robin sequences and complexes: causal heterogeneity and pathogenetic/phenotypic variability. , 1999, American journal of medical genetics.