Fabrication of Sequential Naso-Alveolar Molding Appliances in the Treatment of Cleft Lip/Nose Deformities
Fabrication of Sequential Naso-Alveolar Molding Appliances in the Treatment of Cleft Lip/Nose Deformities
There is a robust body of research suggesting that the use of pre-surgical orthopedic devices
 prior to definitive cleft lip/nose repair results in significant improvement of facial
 aesthetics with long term follow up. However, in recent surveys of the cleft centers in the
 US, only 30% of cleft centers offer PSIOs, and only 13% routinely report its use.
 Accordingly, thirty percent of centers utilize a two-stage cleft lip/nose repair in the
 centers' algorithm (1st: lip adhesion; 2nd: final lip repair). The major drawback to a
 two-stage cleft procedure is the administration of two general anesthetics to an infant
 before the age of one year. There is a growing amount of evidence that multiple anesthetic
 experiences before a certain age could affect brain development.

 It is difficult to make inferences as to why clinicians are not utilizing surgical aids to
 decrease the size of the cleft width, but even when PSIO is offered, caregivers experience
 additional, potentially prohibitive challenges. In one study, caregivers traveled an average
 of 70 miles to visit the nearest cleft center offering pre-surgical orthopedic devices. As
 these devices are created by hand every 1-2 weeks after seeing the child in clinic, parents
 are required to travel to clinic multiple times per month. Not surprisingly, infants who were
 first-born and those who did not have other siblings were more likely to receive pre-surgical
 orthopedic treatment than infants who were residing with other siblings.

 Given the benefits of PSIOs and the barriers both to healthcare systems and patients'
 families associated with PSIOs in its current form, a new form of pre-surgical clinical
 management is needed.

 Objectives:

 1. Evaluate JHH's current clinical performance in addressing unilateral cleft lip and nasal
 deformity.

 2. Elucidate the difference in preoperative cleft size and in surgical management/outcomes
 for patients who received PSIOs through 3D-printed devices.

 3. Using the above maxillofacial growth data with and without PSIOs, the investigators aim
 to create an algorithm to predict maxillofacial growth for each individual patient to
 design pre-sequenced custom PSIO devices.
Approximately 7000 babies are born with a cleft lip and/or cleft palate in the United States
 per year. Globally, this defect is staggeringly common, with 100,000 new cases per year. This
 defect is due to a failure of normal fusion between facial structures, causing a full
 thickness gap of the upper lip involving skin, muscle, and other connective tissue. Due to
 aberrant insertion of the muscle underlying the lip as well as abnormal formation of deeper
 facial structures, the nose is also displaced inferiorly, laterally, and posteriorly. The
 wider the cleft, the more challenging the repair.

 Current best practice in treating unilateral cleft lip and nose deformity involves
 presurgical infant orthopedics (PSIOs). PSIOs involves presurgically treating the deformity
 using custom orthodontics to gradually bring the two halves of the cleft together, reducing
 defect size and enabling complete repair with one surgery during early infancy. However,
 creation of these molds is time consuming and requires the expertise of a cleft orthodontist
 and weekly visits to the hospital for device advancement. As not all cleft centers have a
 specialized cleft orthodontist-including Johns Hopkins Hospital-many are unable to provide
 presurgical infant orthopedics. Weekly travel to cleft centers can also pose a significant
 temporal and financial burden to families, as the average patient is located over 60 miles
 from the nearest cleft center. In a recent survey of cleft centers in the United States, only
 30% of these centers are able to provide presurgical orthopedic molding devices in treatment
 protocols. These centers therefore correct wide clefts with a staged, two-surgery approach.
 This approach is often less successful in both adequately aligning the gum and in repairing
 nasal defects, and usually requires intensive orthodontics and additional surgeries in
 adolescence. As the defect is not fully corrected in infancy, children are additionally
 subjected to the emotional and psychological difficulty and damage to self-esteem associated
 with the persistent defect.

 With the advent of 3D printing technology, the investigators are now in the planning stages
 of implementing a new clinical protocol to bring PSIOs to Johns Hopkins Hospital (JHH). A
 cleft orthodontist at an equipped center will remotely design these orthodontic molds, which
 will then be 3D printed using dental acrylic (the same material currently used in
 hand-fabricated orthodontic models) and given to patients in the investigators' clinic. As
 planning these devices for 3D printing can be done remotely in a fraction of the time it
 would take to create each one by hand, this change in clinical practice will eliminate the
 need for an in-person specialized craniofacial orthodontist on faculty where one does not
 exist.

 This research study seeks to elucidate both the investigators' current standard of cleft
 practice and the benefits the investigators hope to see after implementing this change in
 clinical practice. The investigators expect that although the investigators will initially
 create each new mold after a weekly hospital visit with in-person measurements, the
 investigators ultimately aim to develop an algorithm to predict early growth and development
 of the newborn's facial skeleton and soft tissue. This will allow the fabrication of
 sequential pre-surgical orthopedic molding devices based of the infant's predicted facial
 growth, which can be delivered en bloc to the infants home, thereby eliminating the need for
 frequent visits to geographically inconvenient centers. After 4-5 months of using these
 devices, the bony cleft gap will be closed, the nose straightened, and the child be ready for
 the definitive cleft lip and nose repair in a single surgery. These devices will not only
 physically close the gap of the cleft lip deformity, but will also close the gap in access to
 the necessary specialists for the ideal management of cleft deformities across the country.
 With this technology, any cleft center can offer naso-alveolar molding to every child born
 with cleft lip and palate in the country.
Inclusion Criteria:

 -  All newborns either born in Johns Hopkins or presenting to clinic with a cleft lip
 between 0-1 months of life

 Exclusion Criteria:

 -  Patients older than one month of age at presentation