INTRODUCTION
Ectrodactyly–ectodermal dysplasia–clefting (EEC) syndrome is a rare entity comprising a triad of ectrodactyly, ectodermal dysplasia, and cleft lip with or without cleft palate.[1] The occurrence of all three disorders in one (ectrodactyly, ectodermal dysplasia, and cleft lip/palate) is very rare with a frequency of approximately 1.5/100 million.[1] Mostly, EEC cases are caused by mutations of the TP63 gene and can be either familial (autosomal dominant inheritance) or sporadic (spontaneous mutations).[2]
The feeding difficulties in infants with clefts are well documented in the literature.[3] A feeding appliance basically acts as a “false palate” that restores the separation between the oral and nasal cavities and therefore eases feeding.[4]
However, a feeding plate can be accidentally swallowed, due to which, it is conventionally provided with a thread-like component.[567] The caregivers need to be constantly alert to pull the thread for retrieval of the feeding plate in case of accidental swallowing. The thread also has to be discarded after each use to prevent contamination which is especially relevant in the ongoing pandemic situation since the oral cavity can be an entry portal of severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2).[8] Moreover, certain feeding appliances are provided with retentive hooks; wire components which may cause soft-tissue injury if not handled properly. All of these increase the caregivers' apprehension and reduce patient acceptability.
KRIPA's feeding appliance with its head strap tube-enclosed retentive hook complex may ensure enhanced safety and acceptability. The head strap can also be reused multiple times with easy decontamination.
Herewith, we present a case report that describes the fabrication of KRIPA's feeding appliance for a 3.5-month-old infant affected with EEC syndrome.
CASE REPORT
A 3.5-month-old male infant was referred to our outpatient department with the chief complaint of feeding difficulty. Intraoral examination revealed a unilateral complete cleft lip and palate on the left side [Figure 1]. Extraoral examination revealed ectrodactyly on all limbs. The left foot and part of the left lower leg were absent [Figure 2]. The right foot showed syndactyly between the first, second, third, and fourth toes [Figure 3]. The left hand showed an absence of phalange of the fourth digit and malformed second and fifth digits [Figure 4]. The right hand showed an absence of phalanges of the third digit [Figure 5]. The infant also had sparse brittle hair with persisting hair loss and sparse eyebrows [Figure 6]. After discussion with the child's despondent parents, it was found that the mother was never able to breastfeed the infant and is facing difficulty to feed him even with a long feeding spoon due to nasal regurgitation. Due to the SARS-CoV-2 pandemic, the parents were hesitant in seeking treatment; also the infant did not have optimum body weight for the cleft surgery. Therefore, an immediate decision to fabricate a feeding appliance was made.
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Figure 1: preoperative photograph showing the cleft deformity
Figure 2: left limb shows absent foot and lower leg
Figure 3: right leg shows syndactyly
Figure 4: left hand shows malformed digits and absent phalange
Figure 5: right hand shows absent phalanges
Figure 6: sparse eyebrows
Procedure
An impression of the maxillary arch was made with silicone putty impression material (Dentsply Aquasil Soft Putty) [Figure 7] using a custom-made impression tray [Figure 8] by holding the infant in an upright position. The constant crying of the infant during impression making ensured a patent airway. The impression was poured with dental stone (BNStone, BN Chemicals) [Figure 9]. A 21 gauze orthodontic wire (Jaypee SS Wire) was bent in the shape of two retentive hooks; those hooks were covered with PVC tubes to prevent any soft-tissue trauma. The hooks were attached to the wax spacer over the master cast [Figure 10]. Flexible elastics were tied to the wire hooks by passing them through the eyelets of the hooks. The feeding plate was acrylized with cold cure acrylic resin (DPI) [Figure 11]. The acrylized plate was trimmed, borders were rounded, and polished to avoid trauma to the surrounding tissue. A head strap was made using elastic strap material, buttons, and an adjustable slider in accordance with the infant's head circumference [Figure 12]. The complete feeding appliance was constructed and checked for fit and retention by placing the feeding plate inside the infant's mouth followed by attaching it to the adjustable head strap [Figure 13]. A suckling response was observed by placing a finger inside the infant's mouth. The infant was able to suck and create negative pressure on the finger [Figure 14].
Figure 7: impression
Figure 8: custom-made impression tray
Figure 9: cast
Figure 10: wax spacer with two plastic tube-enclosed retentive hooks
Figure 11: acrylized feeding plate
Figure 12: adjustable head strap
Figure 13: head strap incorporated feeding appliance fitted inside the infant's mouth
Figure 14: suckling response observed
The mother was able to successfully breastfeed the child with the appliance in place. Necessary instructions related to appliance use were given to the parents.
DISCUSSION
Ectrodactyly–ectodermal dysplasia–clefting (EEC syndrome is a rare, complex, pleiotropic multiple congenital anomaly/dysplasia syndrome that requires a multidisciplinary approach for management.[910]
The infant's parents complained of difficulty in feeding the infant. A conventional feeding plate is usually provided with dental floss to prevent gagging or accidental swallowing, but such floss needs to be reused for subsequent feeding approaches.[567] This costs an additional financial burden as well as the fact that the caregivers need to be vigilant enough to pull the floss anytime the plate causes discomfort. Assessing such drawbacks associated with conventional feeding appliances, it was decided to incorporate an adjustable head strap to hold the feeding plate for enhanced protection against accidental swallowing and for a reduction in expenses because the head strap is reusable. The retentive hooks were enclosed with PVC tubes to prevent tissue trauma. As the patient did not undergo presurgical nasoalveolar molding and has surpassed the age for the same at present, utmost precautions were taken during the entire procedure to prevent any injury to the soft-tissue structures. A lubricated gauze piece was inserted into the cleft undercuts to prevent the unnecessary flow of the impression material. Kripa's appliance would aid in proper nutrition resulting in optimal weight gain for the patient to undergo further corrective surgeries. The patient was referred to the multidisciplinary team for further management of the patient's condition. At present, the patient has reported to us stating that her cleft lip repair is done and is awaiting further surgeries and prosthetic rehabilitations as advised by the multidisciplinary team.
CONCLUSION
The management of clinical manifestations associated with EEC syndrome presents a unique challenge. The affected patients suffer physical, psychological, and social distress. KRIPA's feeding appliance is intended to provide a safer yet effective modification to conventional feeding appliances. The term “KRIPA” suggests blessing. KRIPA's feeding appliance aims to improve the quality of life of both the patient and his parents. We look forward to more innovative additions to our humble concept to increase acceptability and awareness about this rare syndrome and its treatment modalities.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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