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A new method of lower lip reconstruction using a full-thickness cheek island flap based on the facial vessels is described. The functional potential of the method is assessed in six patients. The technique outlined is simple in design and execution. It is capable of total lower lip reconstruction without appreciable microstomia. Other major techniques of lip reconstruction are briefly reviewed.
The step technique for lip reconstruction is a simple, flexible, one-stage operation that allows reconstruction of full-thickness defects spanning up to two-thirds of the lower lip and, in our experience, one-half of the upper lip without violating the opposite lip or using distant advancement flaps. Twelve patients, ranging in age from 24 to 86 years, underwent full-thickness lip excisions for squamous cell and deeply invading basal cell carcinomas. Reconstruction was achieved by advancing the remaining lateral lip elements to close the defects in a stepwise fashion without violating the remaining orbicularis oris muscle. All patients had a symmetrical, mobile, oral sphincter with intact commissures, adequate buccal sulcus, no symptomatic microstomia, and normal sensation at the completion of the procedure. There have been no recurrences in a nine-month to four-year follow-up, and satisfactory aesthetic results were achieved.
This study was undertaken to determine whether tangential excision and thick split-thickness skin grafting (STSG) of deep facial burns give a better cosmetic and functional result than conservative management. Forty patients (28 adults, 12 children) treated for facial burns between July 1989 and July 1991 were evaluated in follow-up (mean 18.3 +/- 8.3 months). The patients were categorized into the following groups according to depth and management of their facial burns: (A) healed without surgery in less than 21 days (n = 13), (B) healed without surgery in 21 days or more (n = 11), (C) early debridement and thick STSG in 18 days or less after the burn (n = 6), and (D) delayed debridement and thick STSG in more than 18 days after the burn (n = 10). Facial esthetics were evaluated by use of a modified scar assessment scale [range 0 (normal) to 16 (multiple abnormalities)], and functional problems of the face and neck were evaluated by use of physical examination. Group A patients had a significantly better overall rating on the scar assessment scale (2.1 +/- 2.9) than the patients in the other groups that required more than 21 days to heal, B (8.0 +/- 2.7), C (7.3 +/- 2.9), and D (5.7 +/- 2.5) (p < 0.01, analysis of variance). Also, skin-grafted areas in the surgically treated groups C and D had a significantly better scar rating than wounds that healed spontaneously in group B (5.7 +/- 4.0 vs 8.0 +/- 2.7, p < 0.05; and 5.1 +/- 2.6 vs 8.0 +/- 2.7, p < 0.05). There was no significant difference among groups B, C, and D when the total number of persistent functional problems after treatment were compared. The most common functional problems for these patients were microstomia (17/27) and eyelid ectropion (17/27). Patients with superficial facial burns that heal in less than 21 days appear to heal with generally very acceptable cosmetic and functional results. However, in those patients with deep facial burns that require prolonged periods for spontaneous wound healing, tangential excision of the wound and resurfacing with thick STSG appear to give better cosmetic results than conservative management, with no greater incidence of functional complications.
Facial mutilation after an assault with chemicals is rarely discussed in the literature even though it is a devastating injury that occurs worldwide and is not prohibited or punished by special laws. It is our purpose to describe the devastating outcome of facial mutilation after an assault with chemicals and to draw attention to this injury as a worldwide problem. We studied fifteen patients who sustained facial mutilation with chemicals. The common story was that the patient's spouse was the perpetrator, that sulfuric acid was used, and that the deed occurred after marital or financial discord. All victims were disfigured severely and most became reclusive and rarely left their homes. Six patients (40%) had total bilateral blindness and one suffered partial loss of vision. Lower eyelid ectropion (14), microstomia (12), cervical flexion contracture (10), ear deformity (8), and nostril stenosis (6) were common. Few of the perpetrators were prosecuted. We reviewed the literature and found that the problem has been described all over the world, and that the outcome is similar to that which we described. The problem deserves worldwide recognition and attention.
One hundred one patients with EB were evaluated by a combination of prospective and retrospective review, and analyzed regarding the nature, incidence, and prevalence of their gastrointestinal (GI) manifestations. Involvement of the GI tract is a well-known extracutaneous manifestation of dystrophic EB, but it also occurred in more than one-half and one-third, respectively, of those with junctional and simplex EB. Most of the serious consequences, such as esophageal strictures and microstomia, occurred in recessive dystrophic EB but were also seen, although infrequently, in the junctional and simplex forms. The majority of patients with dysphagia had an esophageal stricture, and the cervical esophagus was the most common location. The onset of dysphagia generally occurred in the first decade of life, in patients much younger than previously recognized. Diagnostic endoscopy did not reveal lesions which could not have been detected radiographically. Lower GI complaints were common, especially constipation and perianal blistering, and affected all types of EB. These complaints contributed substantially to management problems but they did not correlate with colonic pathology and appeared to reflect anal or perianal disease.
A mouth splint has been designed tp be worn through the healing period of an electrical burn of the mouth. The splint is custom-fitted for each child, and it is easily removed. Six children have been so treated and followed up for 4 years. The results to date have been excellent in preventing microstomia.
Maxillectomy defects become more complex when critical structures such as the orbit, globe, and cranial base are resected, and reconstruction with distant tissues becomes essential. This study reviews all maxillectomy defects reconstructed immediately using pedicled and free flaps to establish (1) a classification system and (2) an algorithm for reconstruction of these complex problems. Over a 5-year period, 60 flaps were used to reconstruct defects classified as the following: type I, limited maxillectomy (n = 7); type II, subtotal maxillectomy (n = 10); type IIIa, total maxillectomy with preservation of the orbital contents (n = 13); type IIIb, total maxillectomy with orbital exenteration (n = 18); and type IV, orbitomaxillectomy (n = 10). Free flaps (45 rectus abdominis and 10 radial forearm) were used in 55 patients (91.7 percent), and the temporalis muscle was transposed in five elderly patients who were not free-flap candidates. Vascularized (radial forearm osteocutaneous) bone flaps were used in four of the 60 patients (6.7 percent) and nonvascularized bone grafts in 17 (28.3 percent). Simultaneous reconstruction of the oral commissure using an Estandler procedure was performed in 10 patients with maxillectomy and through-and-through soft-tissue defects. Free-flap survival was 100 percent, with reexploration in five of 55 patients (9.1 percent) and partial-flap necrosis in one patient. Seven of the 60 patients (11.7 percent) had systemic complications, and four died within 30 days of hospitalization. Fifty patients had more than 6 months of follow-up with a mean time of 27.7 (+/- 15.6) months. Postoperative radiotherapy was administered in 32 of these patients (64.0 percent). Chewing and speech functions were assessed in 36 patients with type II, IIIa, and IIIb defects. A prosthetic denture was fixed in 15 of 36 patients (41.7 percent). Return to an unrestricted diet was seen in 16 patients (44.4 percent), a soft diet in 17 (47.2 percent), and a liquid diet in three (8.3 percent). Speech was assessed as normal in 14 of 36 patients (38.9 percent), near normal in 15 (41.7 percent), intelligible in six (16.7 percent), and unintelligible in one patient (2.8 percent). Globe and periorbital soft-tissue position was assessed in 14 patients with type I and IIIa defects. There were no cases of enophthalmos, and one patient had a mild vertical dystopia. Ectropion was observed in 10 of 14 patients (71.4 percent). Oral competence was considered good in all 10 patients with excision/reconstruction of the oral commissure; however, two patients (20 percent) developed microstomia after receiving radiotherapy. Aesthetic results were evaluated at least 6 months after reconstruction in 50 patients. They were good to excellent in 29 patients (58 percent) for whom cheek skin and lip were not resected, and poor to fair (42 percent) when the external skin or orbital contents were excised. Secondary procedures were required in 16 of 50 patients (32.0 percent). Free-tissue transfer provides the most effective and reliable form of immediate reconstruction for complex maxillectomy defects. The rectus abdominis and radial forearm flaps in combination with immediate bone grafting or as osteocutaneous flaps reliably provide the best aesthetic and functional results. An algorithm based on the type of maxillary resection can be followed to determine the best approach to reconstruction.
Scleromyxoedema is a rare disease characterized by cutaneous sclerosis, mucin deposition and paraproteinaemia. Internal disease is common, particularly musculoskeletal, gastrointestinal and central nervous system involvement. We report a series of three consecutive patients with scleromyxoedema treated with high-dose intravenous immunoglobulin (hdIVIg). Each of the three patients had relatively low levels of a highly basic IgG-lambda paraprotein, and each has demonstrated a sustained response of both their cutaneous and extracutaneous disease to hdIVIg. As all patients had perioral skin involvement and microstomia, one measure of cutaneous improvement was the increase in intraincisor distance. Extracutaneous manifestations of scleromyxoedema that improved included ureteral stricture, vocal strength and dysphagia.
Nonsyndromal dysgnathia is a rare disorder with a probable genetic basis characterized by a hypoplastic or absent mandible (agnathia), microstomia, microglossia, and ear anomalies secondary to a defect in the ventral portion of the first branchial arch caused by defective neural crest migration or proliferation. Dysgnathic newborn infants often suffer fatal respiratory failure from airway obstruction. Nineteen children with isolated dysgnathia complex are described in the literature--six were stillborn, eight died shortly after birth, and only five survived infancy. All survivors required tracheostomy to maintain an airway. It is difficult to intubate the trachea of these children and early airway management planning is important. We report a neonate who presented with a prenatal ultrasound diagnosis of severe micrognathia, polyhydramnios and a family history of severe micrognathia. Airway management was achieved with fiberoptic intubation through a laryngeal mask airway (LMA) during an ex utero intrapartum treatment procedure. Fiberoptic intubation was hampered by copious amounts of amniotic fluid. This child and her sibling are the first two siblings with isolated dysgnathia complex to have survived infancy and provide further support for a genetic basis to this condition.
The complications of electrical burn to the oral cavity include microstomia and cosmetic deformity. Surgical burn excision and repair by primary closure, skin grafts, or tongue flaps have recognized disadvantages. The use of intraoral fixed or removable appliances, or the extraoral facemask prosthesis, serves to maintain adequate commissure spacing and allows normal skin and mucosal healing to occur. Secondary commissuroplasties may be required after prosthetic management of the burn.
The frequency of spontaneous anomalies among JW-NIBS rabbits in our laboratory is reported. The study was based on 1217 live fetuses obtained from 185 of an origin sample of 195 pregnant females; the remaining 10 (5.1%) aborted. Seven (0.57%) of the fetuses had the following external anomalies: multiple anomalies with craniofacial anomalies and thoraco-, gastroschisis (3 cases), microcephaly with open eyelids and microstomia (1), microphthalmia (1), anury (1) and brachyury (1). Among 1213 fetuses, 2 (0.16%) had abdominal visceral anomalies: agenesis of gall bladder and hypoplasia of the ovary were each found in one animal. Head and thoracic visceral anomalies were found in 6 (1.73%) of 347 fetuses, and skeletal anomalies in 6 (0.69%) of 867 fetuses. 4.03% of fetuses had 13 ribs.
Scleroderma is a connective tissue disorder which includes fibrosis of the skin. Facial skin contraction leads to loss of forehead, periorbital, and nasolabial wrinkling and at the same time causes radial furrows around the contracted oral opening (microstomia). The lips become retracted. We describe a 41 year old lady whose upper lip was treated by free dermal graft and injection of fat to improve her perioral aesthetic appearance. The skin was taken from the right submammary fold and the fat was obtained from the abdomen by liposuction. Excess fat was stored in a sterile container at -70 degrees C for future use. The patient made a satisfactory recovery and has asked for her lower lip to be treated in the same way.
The authors review the literature on the oculo-mandibulo-facial syndrome and present the case of a six-year-old boy with congenital cataracts, microphthalmos, nystagmus, failure to thrive, dysmorphic features with a tiny pinched nose, mandibular hypoplasia, microstomia, double chin, chronic snoring, recurrent respiratory infections and dental problems. Chronic obstructive sleep apnoea with decreased oxygen saturation was present. Optimal medical management of OMFS-patients is described.
We report on two brothers with low birth weight, growth retardation, microcephaly, minor facial anomalies, mental retardation, and trisomy (6)(p23-->pter) due to a maternal t(6;17)(p23;p13.3). As demonstrated by fluorescent in situ hybridisation (FISH) with the Miller-Dieker cosmid probe (D17S379) and with a subtelomeric probe (D17S34) the additional deletion on 17p13 is very small, and therefore, the phenotype of these two boys is most likely the result of essentially pure partial trisomy 6p. Comparison of the clinical findings with those of ten cases from the literature of dup(6p) with a breakpoint in or more distal to 6p23 allows delineation of a specific phenotype of dup(6)(p23-->pter) characterized by low birth weight, growth retardation, microcephaly, and blepharophimosis, blepharoptosis, microstomia, and abnormal ears.
Cyclophosphamide (CP) is an alkylating agent widely used in treating cancer and autoimmune disease. CP is classified as a pregnancy risk factor D drug and is teratogenic in animals, but population studies have not conclusively demonstrated teratogenicity in humans. Six isolated reports of prenatally exposed infants with various congenital anomalies exist, but to date no specific phenotype has been delineated. The purpose of this report is to document a new case of in utero CP exposure with multiple congenital anomalies and to establish an apparent CP embryopathy phenotype. The mother had systemic lupus erythematosus and cyclophosphamide exposure in the first trimester. She also took nifedipine, atenolol, clonidine, prednisone, aspirin, and potassium chloride throughout pregnancy. The infant had growth retardation and multiple anomalies including microbrachycephaly, coronal craniosynostosis, hypotelorism, shallow orbits, proptosis, blepharophimosis, small, abnormal ears, unilateral preauricular pit, broad, flat nasal bridge, microstomia, high-arched palate, micrognathia, preaxial upper limb and postaxial lower limb defects consisting of hypoplastic thumbs, and bilateral absence of the 4th and 5th toes. Chromosomes were apparently normal. The reported cases of in utero exposure to cyclosposphamide shared the following manifestations with our patient: growth deficiency, developmental delay, craniosynostosis, blepharophimosis, flat nasal bridge, abnormal ears, and distal limb defects including hypoplastic thumbs and oligodactyly. We conclude that (a) cyclophosphamide is a human teratogen, (b) a distinct phenotype exists, and (c) the safety of CP in pregnancy is in serious question.
We present four cases of DiGeorge syndrome diagnosed at our center. Onset occurred during the neonatal period and was associated with severe congenital heart disease. In case 1, the patient had heart disease and absence of thymus. Total T-lymphocytes were 34%; total T4-lymphocytes were 27%. Stimulation test with phytohemagglutinin (PHA), concanavalin A (conA) and pokeweed mitogen were negative. Microdeletion was found in the chromosome 22q11 region. The second case involved heart disease, microstomia, round and rotated ears and branchial cyst. Total T-lymphocytes were 38% and total T4-lymphocytes 27%. Thymus was absent. Microdeletion in the chromosome 22q11 region. Case 3 showed heart disease, renal malformation, absence of thymus and parathyroid gland. The patient died 5 days postsurgery. Microdeletion was seen at chromosome 22q11. In the fourth case there was heart disease, microretrognathia, hypertelorism, short neck, absence of thymus and parathyroid glands. Total T-lymphocytes were 22%, total T4-lymphocytes 15%, and total T lymphocytes for pokeweed mitogen were negative. Microdeletion was found at chromosome 22q11. At the age of 13 days the patient died. The cases were recorded during a 2-year period, between 1997 and 1998. The prevalence of DiGeorge syndrome in the number of admissions for congenital heart disease among the neonates at our hospital was 3.14%. Presentation in the form of repeated infections is rare, since most cases of DiGeorge syndrome are partial, and functional cellular immunity is preserved.
Hereditary epidermolysis bullosa (EB) is a mucocutaneous disorder characterized by the appearance of blisters and vesicles in response to minimum friction. The digestive mucosa is one of the most frequently affected regions--including the oral mucosa. Three types of EB have been established according to the histological level of the lesion. Thus, simple EB involves intraepidermal bullae that leave no scars, while junctional EB exhibit blisters between the lamina lucida and lamina densa of the basal membrane. These lesions heal leaving atrophy and involve important hypoplastic lesions in the dental enamel. In turn, dystrophic EB presents synechiae-forming subepidermal blisters--the recessive form being the variant involving the greatest oral lesions (microstomia, ankyloglossia, milium cysts and rampant caries). Three cases of EB are presented and their clinical-dental management difficulties are described. The oral manifestations are described, along with the dental treatments provided and the evolution of the periodontal indices over a two-year period following the application of hygiene-preventive and therapeutic measures.
Three male sibs from consanguienous parents were found to have a strikingly similar pattern of multiple congenital anomalies. The main features were polyhydramnios; low birthweight; dwarfism; epicanthal folds; abnormal ears; microretrognathia; microstomia; microglossia; glossoptosis; webbed neck; severe cardiac defects; radial and ulnar hypoplasia; radial deviation of the hands; brachymetacarpalia; thumb hypoplasia; clinodactyly and hypoplasia of the 5th finger; simian creases; fibular and tibial hypoplasia; talipes varus with hypoplastic heels; wide gap between 1st and 2nd toes; and delayed bone age. Neonatal death occurred in the 3 babies by severe cardiac failure. Differential diagnosis permits one to conclude that this is a new type of faciocardiomelic dysplasia with a probable autosomal recessive inheritance.