Two cases of single-stage lip and nostril reconstruction in holoprosencephaly

Presurgical naso-alveolar molding paired with cheiloplasty to treat median cleft lip deformity in holoprosencephaly

“…As limited skin and soft tissue volume was available for cheiloplasty, it was quite challenging to reconstruct the lip and nose into a fine structure that forms the cupid bow, philtrum ridge, philtrum dimple, or labial tubercle. The simplest cheiloplasty should involve a straight approximation of the lateral upper lip [2][3][4]. But if a straight scar on the upper lip center is present, the shape of the philtrum structure cannot be determined.…”

“…In some cases, the primary palate and the columella are completely missing, making reconstruction extremely difficult. Various reconstruction methods have been reported based on 'straight-line closure of the upper lip' [2][3][4]. Herein, we report a case of holoprosencephaly with median cleft lip in an infant girl who underwent cheiloplasty and presurgical naso-alveolar molding (PNAM).…”

Holoprosencephaly sequence with a constellation of anomalies in Yenagoa, a Niger Delta Region: report of two cases

“…Previously, it was reported that majority of children with the severe form of HPE rarely survive beyond early infancy hence the treatment for this was symptomatic and supportive. 2,4,17,20 Considering ethical issues regarding surgical beneficence, intervention when carried out should be done at the earliest possible time. 29 However, where the prognosis is very poor, limiting extraordinary medical assistance aimed towards survival is recommended.…”

“…29 However, where the prognosis is very poor, limiting extraordinary medical assistance aimed towards survival is recommended. 2,4,17,29 In the milder forms, since a large number of the children survive past the first year; a multidisciplinary approach to management consist of interventions by plastic surgeons, neurologists, maxillofacial surgeons and psychologists. 2,4,17,24 Unfortunately, the cultural taboos associated with having a child with dysmorphic features influenced the parents to limit care and in one instance, abandon the baby in the hospital.…”

“…13 There are different types of HPE consisting of alobar, semilobar, lobar and middle inter-hemispheric variant (in order of reducing severity). 1,3,4,17 The etiologies of HPE vary ranging from the unknown to multifactorial factors with MRI preferably used to confirm the diagnosis, while Chromosomal microarray (CMA) identifies those with genetic involvement. [3] The treatment is supportive, and requires a multidisciplinary management depending on the degree of forebrain defect.…”