Median Cleft Lip With Pedunculated Skin Masses

Pai syndrome: First patient with agenesis of the corpus callosum and literature review

“… References: 1, Krajina et al, 1965; 2, Das and Majumder, 1970; 3, Sharma, 1974 (case 1); 4, Ponniah, 1977; 5, Iregbulem, 1978 (case 3); 6, Nakamura et al, 1985; 7, Lewin, 1987; 8, Pai et al, 1987; 9, Preece et al, 1988 and Hollis et al, 1996 (case 3); 10, Morgan and Evans, 1990; 11, Reardon et al, 1990; 12, Rudnik‐Shöneborn and Zerres, 1994; 13, Kobayashi et al, 1995; 14, Guion‐Almeida et al, 1996 (case 19); 15, Hollis et al, 1996 (case 1); 16, Masuno et al, 1997; 17, Mishima et al, 1999; 18, Al‐Mazrou et al, 2001; 19, Coban et al, 2003; 20, Szeto et al, 2005; 21, present case. …”

“…Since its first description by Pai and collaborators in 1987, several authors have reported further patients presenting with similar features (Preece et al, 1988; Morgan and Evans, 1990; Reardon et al, 1990; Rudnik‐Shöneborn and Zerres, 1994; Kobayashi et al, 1995; Guion‐Almeida et al, 1996; Hollis et al, 1996; Masuno et al, 1997; Mishima et al, 1999; Al‐Mazrou et al, 2001; Coban et al, 2003; Szeto et al, 2005). In addition, the variable association of MCL, duplicated maxillary median frenulum, and midline facial cutaneous and midanterior alveolar process polyps has been described in at least nine further cases prior to the work of Pai and coauthors (Krajina et al, 1965; Das and Majumder, 1970; Sharma, 1974; Fara, 1977; Ponniah, 1977; Iregbulem, 1978; Nakamura et al, 1985; Scrimshaw, 1986; Lewin, 1987; Gorlin et al, 2001). Despite three recent reviews on the topic (Mishima et al, 1998; Al‐Mazrou et al, 2001; Szeto et al, 2005), the phenotypic spectrum of PS has not yet been entirely elucidated.…”