A Case of Complex Facial Clefts Treated with Staged-tissue Expansion.

SUMMARY: Craniofacial clefts involve all soft tissue and skeletal elements throughout the cleft. Usefulness of tissue expansion in craniofacial clefts is reported. Surgery for a complex type of facial clefts is more difficult and more extensive than for a simple one. We experienced a primary case of complex facial clefts (Tessier No. 2 and 12 on the right and 3, 11, and 5 on the left). Soft-tissue closure of all clefts could be completed by using 4 tissue expanders and 7 operations. Because multiple tissue deficiencies and abnormalities exist in craniofacial clefts, especially complex type, a planned, staged, sequential approach by tissue expansion is necessary to produce ideal results.

Rare craniofacial clefts have been classified by Tessier.[1] Craniofacial clefts include all soft tissues and skeletal elements throughout the cleft. Menard et al[2] reported the usefulness of tissue expansion in a series of Tessier craniofacial clefts.

Surgery for complex facial clefts is more difficult and extensive than for simple ones. Therefore, for complex facial clefts, the use of tissue expansion is recommended. We treated an infant with complex facial clefts (Tessier No. 2 and 12 on the right and 3, 11, and 5 on the left) with staged-tissue expansion.

We decided on the following principles in this case:

Two candidates for tissue expansion were forehead and cheek skin.

We divided the facial clefts into 2 groups: those above the orbit and those below the orbit. Each was reconstructed with a tissue expander in the forehead or cheek, respectively.

We used fewer than 2 expanders per operation because of the patient’s age.

To reduce total operation time, when one was removed, another was placed at a distance.

CASE REPORT

The patient was the second of female twins delivered at 34 weeks and 5 days of gestation. Her sister was normal. At birth, the diagnosis was amniotic band syndrome of the left hand and foot, hydrocephalus, craniosynostosis of the lambda sutures, craniofacial clefts, and right microphthalmos. A ventriculoperitoneal shunt was inserted at 5 days of age. A complete cleft on the left side of the face extended from the lip and undermined the alar base (Tessier No. 3); a bony cleft extended from the left orbital roof (Tessier No. 11) and the cleft of the left upper lip (Tessier No. 5) (Figs. 1, 2).

Fig. 1.

Case: A 4-month-old female infant with complex facial clefts (Tessier No. 2 and 12 on the right and 3, 11, and 5 on the left). Preoperative frontal (A) and basal (B) views.

Fig. 2.

A 3D model of the patient’s facial bones.

A coloboma of the left lower eyelid was present, medial to the punctum. In addition, she had a complete cleft of the right side of the face that extended from the lip to the forehead near the eyebrow. There was an amniotic-like band from the cranial cleft end to the hair line of the forehead (Tessier No. 2 and 12). Because of feeding difficulties and swelling of the exposed nasal cavity mucosa, we planned to close the right cleft and the bilateral cleft lip first. To treat the right Tessier no. 12 cleft and the wide left cleft lip, two 28-cm3 tissue expanders were placed under the skin of the right forehead and the left cheek at the age of 4 months (Table 1). The expanders were inflated with sterile saline, but the expander in the left cheek was exposed into the oral vestibule 2 months after operation, and the next operation was soon performed as the tissue expansion effect persisted. Lambda incisions[3] were performed on the right expanded skin. The bilateral cleft lip was repaired, and the right upper cleft could be closed. A 28-cm3 tissue expander was placed in the forehead to reconstruct the nose. The patient was able to drink milk directly through the mouth after this operation.

Table 1.

Operations

At the third operation at the age of 10 months, 2 lambda incisions were performed on the expanded skin of the forehead (Fig. 3), the nose was advanced, and philtrum-like tissue was fixed to the upper lip. At the fourth operation at 1 year of age, the right malar flap was elevated and advanced to close the right cleft, and the left cleft was closed by an advancement flap. Unfortunately, the right cleft soon reopened postoperatively because of flap tension and nasal drainage due to atresia of the right posterior nasal choana. At the fifth operation at the age of 14 months, a 28-cm3 tissue expander was placed in the right cheek in front of the ear, and a right nostril was constructed.

Fig. 3.

Forehead skin expansion for nasal reconstruction. Two lambda incisions were performed on the expanded skin of the forehead.

At the sixth operation, the cheek flap was advanced again, and the right cleft could be closed. Soft-tissue coverage of the clefts was completed with the use of 4 tissue expanders.

At the seventh operation at the age of 1 year and 9 months, the otolaryngologist made drainage possible from the right nasal cavity to the left nasal cavity by opening the posterior nasal septum to bypass the atresia of the right posterior nasal choana.

At the age of 2 years (Fig. 4), the patient could eat ordinary food, speak jargon, and stand if her hands were held.

Fig. 4.

The patient’s condition at the age of 2 years. Postoperative frontal (A) and basal (B) views.

At 25 months of age, her condition suddenly changed because of acute occlusion of the shunt valve for the hydrocephalus and she went into respiratory arrest. Unfortunately, she died 5 days later.

DISCUSSION

In the first case in 1990, for moving of the left elevated nasal ala in Tessier No. 3, a tissue expander was placed under the forehead skin of a 7-month-old infant. Bony remodeling of the underlying frontal bone was not reported. Remodeling of the bone is one problem of tissue expansion in infants. We also found no remodeling after removing tissue expanders. In the same year, Foley catheters used as tissue expanders were placed over the zygomatic bones of a 14-month-old infant with bilateral oblique facial clefts.[4,5] After removal of the catheters, partial Le Fort II osteotomy and rotation and advance of the cheek flap were performed. In 1994, tissue expansion was used for nasal reconstruction in a patient with a median facial cleft.[6] An intubation tube was used for soft-tissue expansion covering an iliac bone graft.

Menard et al[1] reported tissue expansion in the reconstruction of a series of Tessier craniofacial clefts: primary correction of facial clefts in 8 patients and preparation for bone grafting or osteotomy in 9 patients. They concluded that tissue expansion was effective in tension-free reconstruction for osteotomy and bone grafting. Because multiple tissue deficiencies and abnormalities exist in the complex type of craniofacial clefts, a planned, staged, sequential approach using tissue expansion is necessary to produce ideal results for normal facial growth.

SUMMARY

Craniofacial clefts involve all soft tissues and skeletal elements throughout the cleft. Tissue expansion was useful in the treatment of craniofacial clefts. Surgery for the complex type of facial clefts is more difficult and extensive than for the simple type. We treated an infant with complex facial clefts (Tessier No. 2 and 12 on the right and 3, 11, and 5 on the left). Soft-tissue closure of all clefts could be achieved by using 4 tissue expanders and 7 operations. Because multiple tissue deficiencies and abnormalities exist in craniofacial clefts, especially the complex type, a planned, staged, sequential approach by tissue expansion is necessary to produce ideal results.

PATIENT CONSENT

Parents or guardians provided written consent for the use of the patients’ image.