Macrostomia

Macrostomia is a rare birth defect of the face where the mouth opening is longer and wider than normal, usually stretching toward the ear on one or both sides. Doctors also call it a “transverse facial cleft,” “lateral facial cleft,” or “commissural facial cleft.” In this condition, the normal corner of the mouth (called the oral commissure) is not closed in the usual place, so the mouth looks bigger and sometimes looks like a cut going sideways on the cheek. [1] Macrostomia happens before birth, when the baby’s face is forming in the womb. During early pregnancy, parts of the upper jaw (maxillary process) and lower jaw (mandibular process) should grow toward each other and join. In macrostomia, this joining (fusion) is not complete, so an opening stays at the side of the mouth. [2]

Macrostomia is a rare birth difference in which the corner of the mouth (oral commissure) is opened wider than normal, often extending sideways toward the ear. Doctors also call it a transverse facial cleft or Tessier 7 cleft. In some children, only the skin is involved; in others, the muscles of the lips and even the bones of the cheek and jaw can be affected. This can cause problems with feeding, drooling, speech, and facial appearance, so treatment usually involves careful surgery in early childhood to rebuild the corner of the mouth and the lip muscles. [PubMed]

Macrostomia may occur on one side of the face (unilateral) or both sides (bilateral). The cleft often runs horizontally or slightly upwards toward the ear and may be associated with other facial differences such as ear anomalies or hemifacial microsomia. Because the lips and facial muscles are important for sucking, blowing, and speaking, children with macrostomia can have difficulty feeding as infants and may later develop speech problems or social anxiety about their appearance if it is not corrected. A specialized craniofacial team usually plans surgery so that the new mouth corner is symmetrical and the scars hide in natural skin lines. [NIH]

Doctors group macrostomia as part of the “Tessier number 7 facial cleft,” which is one of the rare lateral (side) facial clefts in the Tessier cleft classification system. This cleft runs from the mouth toward the small ear cartilage in front of the ear (tragus) and may involve skin, muscles, and sometimes bone of the cheek and jaw. [3]

Macrostomia is uncommon. Studies report it in about 1 in 60,000 to 1 in 300,000 live births. It may affect one side of the face (unilateral) or both sides (bilateral). In many reports, boys seem to be affected a little more often than girls. [4]

Macrostomia can appear alone (isolated) or together with other problems of the face, ears, eyes, or bones. It is often seen as part of wider craniofacial conditions, such as hemifacial microsomia and Goldenhar syndrome, where one side of the face is under-developed. [5]

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Other names

Doctors use several other names that mean almost the same thing as macrostomia. Common terms are “transverse facial cleft,” “lateral facial cleft,” and “commissural facial cleft.” These names all describe an opening that runs sideways from the corner of the mouth into the cheek. [6]

Macrostomia is also the key soft-tissue feature of “Tessier number 7 cleft,” which is the code used in the Tessier craniofacial cleft classification. In some papers, doctors simply write “Tessier 7 cleft with macrostomia,” especially when bone of the jaw and cheek is also abnormal. [7]

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Types of macrostomia (list view)

Doctors describe a spectrum (range) of macrostomia types, from very mild to very severe. [8]

1. Isolated macrostomia – wide mouth without other major face or body problems.

2. Unilateral macrostomia – only one side of the mouth is widened.

3. Bilateral macrostomia – both sides of the mouth are widened. [9]

4. Simple (partial) transverse facial cleft – the cleft goes only up to the front edge of the chewing muscle (masseter); muscles and bones are mostly normal. [10]

5. Complete transverse facial cleft – the opening extends from the mouth almost to the ear area, with skin, muscle, and sometimes bone missing or misplaced. [11]

6. Macrostomia with mainly soft-tissue involvement – only skin and muscles are affected; bones are normal. [12]

7. Macrostomia with skeletal (bone) involvement – jaw and cheek bones (maxilla and zygoma) are also malformed or under-developed. [13]

8. Syndromic macrostomia – macrostomia occurs as part of a known syndrome (for example Goldenhar syndrome, Treacher Collins syndrome, Ablepharon-macrostomia syndrome). [14]

9. Asyndromic macrostomia – macrostomia is present without any clear named syndrome or other major system problems. [15]

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Causes of macrostomia

1. Failure of fusion of maxillary and mandibular processes
   In early pregnancy, the upper jaw part (maxillary process) and lower jaw part (mandibular process) should grow toward each other and fuse at the future corner of the mouth. In macrostomia, this fusion fails or is incomplete, so a gap remains and the mouth opening is wider than normal. [16]

2. Abnormal migration of facial mesoderm cells
   During face development, a group of cells called mesoderm must move into the first and second branchial arches to form muscles and soft tissues. If this movement is faulty, the tissues at the mouth corner may be too thin or absent, leading to macrostomia. [17]

3. Isolated genetic factors (familial cases)
   In some families, more than one person has macrostomia, suggesting a genetic cause even when a full syndrome is not diagnosed. These genetic changes may affect how the facial processes grow and fuse, although exact genes are not fully known. [18]

4. Hemifacial microsomia / craniofacial microsomia
   Hemifacial microsomia is a condition where one side of the face is under-developed. Macrostomia is common in this setting, because the lower face and ear region formed from the first and second branchial arches do not grow and fuse normally. [19]

5. Goldenhar syndrome (oculo-auriculo-vertebral spectrum)
   Goldenhar syndrome involves abnormal development of the ears, eyes, spine, and one side of the face. Macrostomia and lateral facial clefts are often described as part of this spectrum, due to disturbed growth of the first and second branchial arches. [20]

6. Treacher Collins syndrome
   Treacher Collins syndrome is a genetic condition with under-developed cheek bones, jaw, and ears. Some patients show lateral facial clefts or macrostomia because the cheek and jaw tissues do not form or fuse normally. [21]

7. Apert syndrome
   Apert syndrome is a craniosynostosis syndrome with early skull bone fusion and midface changes. Macrostomia has been reported in some Apert cases, likely due to disturbed facial bone and soft-tissue development. [22]

8. Crouzon syndrome
   Crouzon syndrome also causes early skull bone fusion and midface deformity. Lateral facial clefts or macrostomia can appear in this setting when the maxillary and mandibular segments are mis-aligned during growth. [23]

9. Ablepharon-macrostomia syndrome
   In this rare syndrome, the eyelids are poorly formed or absent (ablepharon), and the mouth is abnormally wide (macrostomia). The cause is usually a specific genetic change affecting skin and craniofacial development. [24]

10. Simpson-Golabi-Behmel syndrome
    This overgrowth syndrome can include facial clefts and macrostomia along with large body size and organ anomalies. The underlying gene changes disturb normal growth signals, including those in the facial region. [25]

11. Fryns syndrome
    Fryns syndrome is a multiple-anomaly condition with diaphragmatic hernia and craniofacial defects. Lateral facial clefts and macrostomia are listed among the possible facial features, again due to very early errors in facial development. [26]

12. Barber–Say syndrome
    Barber–Say syndrome is another very rare genetic disorder with loose skin and facial anomalies. Macrostomia has been described in reported cases, pointing to the effect of the gene defect on mouth and cheek formation. [27]

13. Other rare craniofacial syndromes
    Some other named and unnamed craniofacial syndromes can include macrostomia as one of several facial clefts or asymmetries. In these cases, many structures of the head and neck form abnormally, and the side of the mouth is just one part of the pattern. [28]

14. Vascular disruption during early facial development
    Some authors suggest that reduced blood flow to the face in early pregnancy may damage growing tissues in the branchial arches. This vascular problem could prevent the normal closing of the mouth corner and lead to a lateral cleft. [29]

15. Exposure to certain drugs in early pregnancy
    A few case series link facial clefts, including macrostomia, to exposure to strong teratogenic medicines in early pregnancy, such as high-dose retinoic acid. These drugs can interfere with signalling pathways that guide facial growth. Evidence is limited, but it suggests a possible risk factor. [30]

16. Maternal metabolic diseases (for example, diabetes)
    Poorly controlled diabetes and some other maternal metabolic disorders are known risk factors for many birth defects. They may increase the risk of craniofacial malformations, including unusual facial clefts, through effects on blood vessels and developing cells. [31]

17. Chromosome micro-deletions (for example, 22q11.2)
    Some reports show craniofacial anomalies and hemifacial microsomia with small missing pieces of chromosome 22 (22q11.2 deletion) and other chromosomal changes. These micro-deletions can disturb genes important for branchial arch development and may be linked to macrostomia in some children. [32]

18. Mechanical factors (for example, amniotic bands)
    Very rarely, bands of tissue in the womb (amniotic bands) or unusual pressure on the baby’s face may distort the developing cheek and mouth region. This mechanical force could contribute to facial clefts, including lateral clefts, though strong evidence is limited. [33]

19. Environmental toxic exposures
    Exposure to certain toxins, chemicals, or heavy metals early in pregnancy has been connected in some studies to facial malformations in general. These agents may harm rapidly dividing cells in the facial processes and contribute to macrostomia in a multifactorial way. [34]

20. Multifactorial and unknown causes
    In many children with macrostomia, no single clear cause is found. Most experts think that genes and environment usually act together. A small disturbance during a short, critical time window of facial development may be enough to prevent normal fusion of the mouth corner. [35]

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Symptoms and signs of macrostomia

1. Unusually wide mouth opening
   The most obvious sign is a mouth that is longer side-to-side than normal. The opening may extend toward the ear, and the line between lip and cheek may look broken or pulled. [36]

2. Abnormal or missing mouth corner (oral commissure)
   The normal sharp corner of the mouth may be flat, rounded, split, or missing. Sometimes the corner is pulled outward or upward along a small groove in the cheek. [37]

3. Facial asymmetry
   When only one side is involved, the face may look uneven. One side of the mouth may be longer or lower than the other, especially when the child smiles or cries. [38]

4. Problem fully closing the mouth
   Because the lips are not joined in the normal place, the child may not be able to close the mouth tightly. Small gaps can remain at the side, especially during sleep. [39]

5. Drooling and saliva leakage
   Saliva can leak out of the side cleft, causing drooling and wetness of the cheek. This can irritate the skin and may become socially embarrassing as the child grows. [40]

6. Feeding difficulty in newborns and infants
   Babies may have trouble making a tight seal around the breast or bottle. They may lose milk from the side of the mouth, take a long time to feed, or tire easily during feeds. [41]

7. Chewing problems in older children
   As children start solid foods, they may have difficulty chewing and keeping food inside the mouth. Food may escape from the cleft area, making eating messy and tiring. [42]

8. Speech and articulation problems
   Some children may find it hard to form certain sounds that need firm lip closure, such as “p,” “b,” and “m.” Air may escape from the side cleft, and speech may sound unclear or slurred until after repair and speech therapy. [43]

9. Facial expression difficulty
   Because the muscles around the mouth may be split or misplaced, the child may have trouble smiling evenly, puffing out the cheeks, or whistling. Expressions can look lopsided. [44]

10. Dental and bite problems (malocclusion)
    In cases with bone involvement, the upper and lower teeth may not meet properly. This malocclusion can cause chewing problems and may need orthodontic care later. [45]

11. Skin irritation or infections at the cleft edge
    Continuous wetness from saliva and friction from movement can cause redness, soreness, or small infections at the edge of the cleft or scar area. [46]

12. Ear abnormalities and hearing problems (in syndromic cases)
    When macrostomia is part of syndromes like Goldenhar or Treacher Collins, the child may have small or misshapen ears, skin tags in front of the ear, or hearing loss. These problems can add to communication difficulties. [47]

13. Eye anomalies (in syndromic cases)
    Some related syndromes cause eye problems such as dermoid growths on the eye surface or abnormal eyelids (for example in Ablepharon-macrostomia syndrome). These may reduce vision or cause eye dryness. [48]

14. Associated skeletal or spinal problems (in syndromic cases)
    In Goldenhar and related conditions, vertebral (spinal) anomalies and rib changes can be present along with macrostomia. These may cause posture problems or back pain later in life. [49]

15. Psychosocial and emotional impact
    Visible facial differences can affect self-confidence, social interactions, and mental health. Children and teenagers with macrostomia may feel shy, be teased, or avoid social events unless they receive support and, when needed, reconstructive surgery. [50]

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Diagnostic tests for macrostomia –

Physical examination

1. Newborn and general physical examination
   Diagnosis usually starts with a careful exam right after birth. The doctor inspects the baby from head to toe, notes the wide mouth opening, checks for other facial clefts, and looks for problems in the ears, eyes, spine, heart, or limbs that might suggest a syndrome. [51]

2. Detailed facial inspection at rest and during movement
   The clinician looks at the face from the front and from the side, both when the child is still and when the child cries, smiles, or talks. This helps to see the exact length and direction of the cleft and to judge how the lips meet and move. [52]

3. Oral cavity and dental examination
   The inside of the mouth, gums, and teeth are examined. The doctor or dentist looks for clefts inside the cheek, abnormal gum lines, missing or extra teeth, and how the upper and lower jaws fit together. [53]

4. Assessment of lip competence and saliva control
   The examiner checks whether the child can keep the lips closed at rest and during swallowing. They watch for saliva pooling or leaking from the cleft, which helps plan the degree of surgical correction needed. [54]

5. Feeding observation test
   In babies, a nurse or speech-language therapist may watch a full feed. They note how well the baby latches, how much milk is lost, how long the feed takes, and whether the baby shows signs of tiredness or breathing difficulty during feeding. [55]

6. Ear and hearing examination
   The outer ears are inspected for shape and position, and the ear canal is checked. Newborn hearing screening or formal hearing tests are done, especially if there are ear tags, small ears, or a known syndrome. [56]

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Manual function tests

7. Facial muscle function test
   In older infants and children, the clinician asks the child to smile, frown, puff out the cheeks, close the eyes tightly, and show the teeth. These actions test the strength and coordination of the facial muscles, and show how much the cleft affects movement. [57]

8. Jaw range-of-motion test
   The child is asked to open and close the mouth as wide as possible and move the jaw to each side. The doctor measures how far the jaw moves and checks for pain or locking, which may point to bone or joint involvement in the cleft. [58]

9. Chewing and bite function assessment
   For children who eat solid foods, clinicians may watch the child chew different food textures. They observe whether food stays inside the mouth, how the teeth meet, and whether the child avoids chewing on the affected side. [59]

10. Speech and articulation assessment
    A speech-language therapist listens to the child’s speech and checks how the lips, tongue, and soft palate work together. They note which sounds are hard to produce and whether air escapes at the side of the mouth, helping to plan therapy and timing of surgery. [60]

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Lab and pathological tests

11. Basic blood tests (complete blood count and biochemistry)
    Basic blood tests are usually normal in isolated macrostomia but may be done to look for other health issues, to prepare for surgery, or to check for organ problems when a wider syndrome is suspected. [61]

12. Genetic consultation and gene panel testing
    If the child has other anomalies (ear, eye, spine, limb, or organ problems), a clinical geneticist may order gene panel tests for craniofacial syndromes such as Treacher Collins, Goldenhar spectrum, or Ablepharon-macrostomia syndrome. The goal is to find an exact genetic cause. [62]

13. Chromosomal microarray and targeted tests (for example, 22q11.2 deletion)
    A chromosomal microarray can detect small missing or extra pieces of chromosomes. When facial asymmetry and other systemic problems are present, tests for deletions like 22q11.2 may be considered, because they are linked with several craniofacial conditions. [63]

14. Pathology of associated skin or soft-tissue lesions
    If there are pre-auricular skin tags, dermoids, or other unusual growths, a surgeon may remove them and send them for histopathology. The lab report can confirm associated conditions such as dermoid cysts in Goldenhar syndrome. [64]

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Electrodiagnostic tests

15. Facial nerve conduction studies
    In complex or unclear cases, doctors may use nerve conduction tests to see how electrical signals travel along the facial nerve. This helps to understand if weakness around the mouth is due only to the cleft structure or also to nerve damage. [65]

16. Electromyography (EMG) of facial muscles
    EMG uses fine needles or surface electrodes to measure activity in facial muscles at rest and during movement. It can show whether muscles at the mouth corner are properly formed and working, which can guide surgical repair planning. [66]

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Imaging tests

17. Plain radiographs (X-rays) of the face and skull
    Simple X-rays can give an overview of the skull and jaw bones. They may show asymmetry, missing parts of the cheek bone, or abnormal jaw shape, especially in children with syndromic macrostomia. [67]

18. Computed tomography (CT) scan with 3D reconstruction
    CT scans offer detailed, three-dimensional images of the bones of the face, including the maxilla, mandible, and zygoma. They are very useful to see how far the Tessier 7 cleft extends into bone and to plan osteotomies or other skeletal corrections. [68]

19. Magnetic resonance imaging (MRI) of head and neck
    MRI gives clear pictures of soft tissues such as muscles, nerves, and the brain without radiation. It may be used when doctors suspect deeper soft-tissue involvement, brain anomalies, or when planning complex craniofacial surgery. [69]

20. Prenatal ultrasound and fetal MRI
    In some pregnancies, a skilled sonographer can see a wide mouth or lateral facial cleft on routine ultrasound. Fetal MRI can give extra details about the facial structures and help parents and doctors plan care before and after birth. [70]

Non-pharmacological treatments

1. Parent education and psychological support
Before and after surgery, counselling helps parents understand the condition, the surgical plan, and realistic results. The purpose is to reduce anxiety, improve bonding with the baby, and encourage consistent home care. Psychologists and social workers may teach coping skills, explain how facial differences can affect self-esteem, and help prepare the child for school and social situations. This emotional support improves long-term quality of life and helps families stick with follow-up visits and therapies. [Craniofacial Team]

2. Feeding assessment and specialized bottles
In early infancy, a speech or feeding therapist evaluates how well the baby can suck, swallow, and coordinate breathing. The purpose is to prevent choking, aspiration, and poor weight gain. Special bottles, soft nipples, or altered positions can help the baby form a better seal around the mouth and direct milk safely toward the throat. This non-drug approach can avoid the need for feeding tubes and ensure good nutrition until surgery is done. [Feeding Therapy]

3. Positioning and handling techniques
Careful positioning during feeding and sleep can reduce drooling, choking episodes, and reflux. The purpose is to keep the airway safe and make feeding less tiring. Parents may be taught to hold the baby slightly upright, support the chin, and avoid flat lying immediately after feeds. These simple measures improve comfort and may lessen the risk of respiratory complications. [Pediatric Surgery Care]

4. Speech and language therapy
As the child grows, a speech-language pathologist evaluates articulation, language development, and resonance. The purpose is to identify early speech problems linked to lip muscle weakness or abnormal mouth shape. Play-based exercises, sound practice, and breathing control activities help the child use the reconstructed lips efficiently and improve speech clarity, reducing frustration and social embarrassment. [Speech Therapy]

5. Orofacial myofunctional therapy
This therapy focuses on the muscles of the lips, cheeks, and tongue. The purpose is to improve lip seal, control saliva, and normalize oral habits such as mouth breathing or tongue thrusting. Gentle exercises, blowing games, and resistance tasks train the repaired orbicularis oris muscle to work symmetrically, helping with eating, drinking from a cup, and producing certain sounds. [Myofunctional Therapy]

6. Scar massage and topical care
After surgery, regular scar massage with approved ointments can soften the scar, improve flexibility, and reduce tightness at the mouth corner. The purpose is to minimize contracture that could pull the commissure out of position. Parents are shown gentle circular movements and stretching techniques. Silicone gels or sheets may also be used to flatten and fade scars, improving cosmetic results. [Scar Management]

7. Sun protection for facial scars
Protecting the surgical scar from sunlight using hats, clothing, or child-safe sunscreen reduces the risk of dark, noticeable scars. The purpose is purely cosmetic but hugely important for self-confidence. Newly healed scars are very sensitive to UV light; good protection during the first year helps them blend with the surrounding skin and remain less visible during facial expressions. [Dermatology Guidance]

8. Child-friendly pain coping techniques
Non-drug methods such as distraction with toys, music, breathing exercises, and comfort from caregivers are important after surgery. The purpose is to reduce perceived pain and anxiety while lowering the need for strong medicines. Studies in pediatric surgery show that combined non-pharmacological and non-opioid approaches can effectively manage pain and improve recovery. [Pediatric Pain Control]

9. Physical therapy for facial movement
Some children benefit from guided exercises to improve symmetry of facial expressions and lip movements. The purpose is to strengthen weak muscles, prevent stiffness, and support coordinated smiling, blowing, and chewing. Therapists may use mirror feedback and play to encourage equal use of both sides of the face, especially after unilateral macrostomia repair. [Rehabilitation]

10. Behavioral strategies to manage drooling
Drooling can be socially distressing. Therapists may teach the child to recognize when saliva builds up and swallow more often, use reminders, or keep a small towel or bib discreetly. The purpose is to improve social comfort and hygiene without medications. Over time, as muscle control improves and habits change, drooling often decreases. [Behavior Therapy]

11. Occupational therapy for feeding skills
Occupational therapists can work on how the child handles utensils, cups, and different food textures. The purpose is to make mealtimes safe and age-appropriate despite prior mouth surgery. Practice with soft, then gradually firmer foods, plus adaptive utensils when needed, builds confidence and independence in eating. [Occupational Therapy]

12. Dental and orthodontic monitoring
Regular visits to pediatric dentists and orthodontists are essential because jaw and tooth development may be affected. The purpose is to detect malocclusion, tooth crowding, or enamel issues early and plan braces or other treatments at the right time. Good oral hygiene instruction also prevents cavities around the scarred areas where cleaning can be more difficult. [Pediatric Dentistry]

13. Multidisciplinary craniofacial team follow-up
A craniofacial team (surgeon, orthodontist, speech therapist, psychologist, etc.) reviews the child periodically. The purpose is to adjust care as the face grows, decide if revision surgery is needed, and support schooling and social integration. Such coordinated follow-up is considered best practice in rare facial clefts, improving both function and aesthetics over time. [Craniofacial Center]

14. School and social support interventions
Teachers and classmates may need basic information so they respond kindly rather than with teasing. The purpose is to protect mental health and encourage inclusion. School counsellors can help the child build social skills and confidence, while anti-bullying policies ensure a safer environment. This psychosocial support is as important as the physical repair in long-term outcomes. [Psychosocial Care]

15. Nutritional optimization for wound healing
Dietitians can assess calorie, protein, vitamin, and mineral intake, especially around surgery. The purpose is to support good wound healing and growth. Adequate protein, vitamin C, zinc, and overall energy are known to help collagen formation and tissue repair, so the care team may adjust foods or formulas to meet these needs. [Clinical Nutrition]

16. Early intervention developmental programs
If macrostomia is part of a broader syndrome, early intervention services may provide physical, occupational, and speech therapy in one program. The purpose is to support overall development—motor skills, language, and social interaction—during the critical first years of life. Early therapy can reduce long-term delays and help the child reach school readiness. [Early Intervention]

17. Sleep and airway monitoring
Some children with craniofacial anomalies have snoring or breathing issues during sleep. The purpose of non-invasive monitoring, sleep studies when needed, and positional strategies is to ensure safe breathing and good rest, which indirectly support healing and daytime behavior. Parents may be taught to watch for labored breathing and unusual sleep positions. [Sleep Medicine]

18. Home wound care education
Nurses educate caregivers about cleaning the incision, recognizing infection signs, and knowing when to call the team. The purpose is to prevent complications and avoid unnecessary emergency visits. Clear written instructions, photos, and checklists help families feel confident caring for delicate facial wounds at home. [Postoperative Care]

19. Use of soft protective dressings
Short-term use of soft dressings or adhesive strips can protect the incision from accidental rubbing and keep tension off the scar. The purpose is to promote neat healing and reduce scar spread. Care teams choose dressings that are gentle on infant skin and teach families how long to use them and how to change them safely. [Surgical Nursing]

20. Regular photographic and clinical documentation
Taking standardized photos and clinical notes at each visit helps the team track growth, scar changes, and facial symmetry. The purpose is to guide decisions about minor revisions or further therapy at the right age. This careful documentation also reassures families by showing before-and-after progress over time. [Clinical Audit]

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Drug treatments around macrostomia surgery

There is no medicine that “cures” macrostomia itself. The cleft is a structural difference, so surgery is the main treatment. Medicines are used to support surgery by controlling pain, preventing infection, reducing nausea, and supporting healing. All dosing must be decided by pediatric anesthesiologists and surgeons; parents and patients should never adjust doses on their own. [Craniofacial Surgery]

Below are examples of commonly used drug classes, with evidence from pediatric pain and antibiotic guidelines and [FDA prescribing information] for individual products, not specific “macrostomia drugs.”

1. Intravenous acetaminophen (paracetamol)
IV acetaminophen is a core non-opioid analgesic used immediately after surgery to reduce pain and fever. It works mainly by blocking pain-related enzymes in the brain. It allows lower opioid doses and smoother recovery in children. Pediatric IV dosing is weight-based and carefully limited to prevent liver toxicity; details are described in the [OFIRMEV FDA label].

2. Oral acetaminophen
As soon as the child can drink safely, oral acetaminophen often replaces IV forms. The purpose is to maintain steady background pain control at home and in the ward. It has a long safety history in children when used within weight-based limits and is recommended in many pediatric pain protocols as first-line therapy for post-surgical pain. [Guidelines]

3. Non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen
NSAIDs reduce pain and inflammation by blocking cyclooxygenase enzymes that produce prostaglandins. In craniofacial and cleft surgeries, combining acetaminophen with ibuprofen can provide strong pain relief and reduce opioid needs. Pediatric protocols specify careful dosing and avoidance in children with kidney disease or bleeding risk. [Ibuprofen Injection FDA label] and pediatric pain guidelines describe these precautions.

4. Local anesthetic nerve blocks (e.g., bupivacaine, lidocaine)
During surgery, surgeons or anesthetists may inject local anesthetics near sensory nerves around the mouth. The purpose is to block pain signals at their source for several hours after surgery, reducing the need for systemic opioids. These drugs stabilize nerve membranes and prevent transmission of pain impulses. Their use is standard practice in many pediatric facial procedures. [Regional Anesthesia]

5. Short-term opioids (e.g., morphine, fentanyl) in monitored settings
For major reconstructions, some children may still need small doses of opioids for breakthrough pain under close monitoring. Opioids work by binding to μ-receptors in the brain and spinal cord to blunt pain perception. Because of risks such as sedation and respiratory depression, guidelines stress careful dosing, brief use, and ready access to naloxone as an antidote. [Opioid Safety]

6. Perioperative antibiotics (e.g., ampicillin–sulbactam)
Surgeons may give a single IV dose of an antibiotic just before incision to lower the risk of surgical site infection, especially in mouth surgeries where bacteria are abundant. Ampicillin–sulbactam is one commonly used agent; its pediatric IV dosing and safety profile are detailed in the [UNASYN FDA label]. The choice and need for prophylaxis depend on local protocols and individual risk.

7. Topical antibiotic ointments
After surgery, some teams prescribe short-term topical antibiotic ointments for stitches at the lip to lower surface infection risk and keep the wound moist. These products act locally on skin bacteria with minimal systemic absorption. Their use should follow local infection-control guidelines to avoid unnecessary antibiotic exposure. [Topical Therapy]

8. Antiemetic drugs (e.g., ondansetron)
Nausea and vomiting after anesthesia can strain the new mouth repair and risk wound opening. Antiemetics like ondansetron block serotonin receptors involved in the vomiting reflex. They are often given around the time of surgery to keep the child comfortable and protect the sutures. Pediatric dosing and safety are provided in [FDA prescribing information] and anesthesia guidelines.

9. Perioperative corticosteroids (e.g., dexamethasone)
Low-dose steroids may be used during anesthesia to decrease swelling and reduce postoperative nausea. They work by dampening inflammatory pathways and stabilizing blood vessels. In craniofacial surgery, careful steroid use can improve comfort, but doses and timing are strictly controlled by the anesthesiologist to minimize side effects like immune suppression or high blood sugar. [Anesthesia Guidelines]

10. Multimodal combination products (acetaminophen + ibuprofen)
Some newer IV or oral products combine acetaminophen and ibuprofen in a fixed ratio to simplify dosing and enhance pain control. FDA labels for these products provide detailed, weight-based pediatric doses and warn against exceeding daily limits of either component. The mechanism is complementary: central analgesia from acetaminophen plus peripheral anti-inflammatory effects from ibuprofen. [FDA Label]

(Because macrostomia is rare and treated surgically, evidence-based drug use focuses on these general pediatric perioperative medications rather than disease-specific drugs.)

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Dietary molecular supplements (supportive, not curative)

There are no supplements proven to “fix” macrostomia, but good nutrition supports growth, immunity, and wound healing after surgery. All supplements must be supervised by a pediatrician or dietitian, especially in young children.

1. Vitamin C
Vitamin C is essential for collagen production, immune defense, and antioxidant protection. After surgery, adequate vitamin C from foods or medically guided supplements can support normal wound healing and reduce the chance of delayed repair. The mechanism involves helping fibroblasts build collagen and limiting oxidative stress at the incision site. [Vitamin C – Wound Healing]

2. High-quality protein supplements
If children struggle to eat enough solid food after surgery, protein-rich formulas or shakes may be used. Protein provides amino acids needed for tissue repair, immune cell production, and growth. The mechanism is simple: more available amino acids for building new collagen, muscle, and enzymes during the healing phase. [Protein and Wounds]

3. Arginine-enriched formulas
Some perioperative formulas are enriched with arginine, an amino acid that supports nitric oxide production, blood flow, and immune function. Studies in surgical patients suggest that arginine-containing formulas may improve certain wound-healing outcomes, though evidence in children is still limited. The mechanism involves improved perfusion and collagen deposition. [Arginine Supplementation]

4. Glutamine supplementation
Glutamine is a conditionally essential amino acid during stress and surgery. It serves as fuel for rapidly dividing cells in the gut and immune system. Some studies show that glutamine may support recovery after major surgery by improving nitrogen balance and possibly aiding wound healing. It should only be used when clearly indicated by clinicians. [Glutamine and Wounds]

5. Omega-3 fatty acids (fish oil)
Omega-3 fatty acids from fish oil have anti-inflammatory properties and may modulate the wound-healing response. They can reduce excessive inflammation and possibly improve epithelialization, although timing and dose are important, and data in children are mixed. The mechanism involves altering production of pro-inflammatory and pro-resolving lipid mediators. [Omega-3 and Healing]

6. Zinc
Zinc is a trace mineral necessary for DNA synthesis, immune function, and epithelial repair. Deficiency can slow wound closure; correcting low zinc levels may help normal healing after facial surgery. However, excessive zinc can interfere with other minerals such as copper, so any supplementation must be guided by a clinician. [Zinc and Wounds]

7. Vitamin D
Vitamin D supports bone health, immune function, and muscle strength. Ensuring adequate vitamin D may help overall recovery and resistance to infection after craniofacial surgery, particularly in children with limited sun exposure. Its mechanism includes modulation of immune cells and calcium metabolism. [Vitamin D]

8. Probiotics
Probiotics are live beneficial bacteria that may support gut health and immune balance, especially when antibiotics are used around surgery. By stabilizing the intestinal microbiome, they may reduce antibiotic-associated diarrhea and indirectly support nutritional status and healing. Choice of strain, dose, and duration must follow pediatric guidance. [Probiotics]

9. Multivitamin/mineral supplements
When dietary intake is poor, a standard pediatric multivitamin/mineral can help cover basic needs for vitamins A, B-complex, C, D, E, and trace elements. The mechanism is broad support of many enzyme systems involved in tissue repair and immune defense, rather than any direct action on macrostomia. Care teams avoid mega-doses and tailor supplements to age and diet. [Micronutrients]

10. Specialized “immunonutrition” drinks
Some hospital-supervised drinks combine protein, arginine, omega-3 fatty acids, and β-hydroxy-β-methylbutyrate (HMB) to support recovery from major surgery. Evidence in adult high-risk operations suggests these formulas may reduce complications; pediatric data are still emerging. They are used only under specialist direction for selected cases. [Immunonutrition]

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Immunity-booster and regenerative / stem-cell–related therapies

At present, there are no approved “stem cell drugs” or specific immune-booster medications for macrostomia. Research in craniofacial surgery explores tissue-engineered grafts, stem-cell–enriched fat grafting, and advanced biomaterials to improve soft-tissue and bone reconstruction, but these approaches are experimental and used only in clinical trials or highly specialized centers. [Craniofacial Research]

For now, the most effective “immunity and regeneration support” for a child with macrostomia is:

• Routine vaccines according to national schedules.

• Good nutrition and growth monitoring.

• Prompt treatment of infections.

• Carefully planned reconstructive surgery by an experienced team.

Any advertisement of “stem-cell pills” or unregulated injections for congenital facial clefts is not evidence-based and may be dangerous. Families should discuss only scientifically supported options with their craniofacial team. [Regulatory Guidance]

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Surgeries for macrostomia

1. Commissuroplasty (reconstruction of the mouth corner)
Commissuroplasty is the core procedure in macrostomia repair. The surgeon designs a new mouth corner at the correct position by mirroring the normal side, then reconstructs the orbicularis oris muscle and mucosa. The purpose is to restore a functional, symmetrical commissure that allows normal lip closure, speech, and expression. It is usually done in infancy or early childhood once the child is fit for anesthesia. [Commissuroplasty]

2. Z-plasty skin closure
In Z-plasty, triangular skin flaps are rearranged in a Z-shaped pattern around the new mouth corner. The purpose is to break up the scar line, reduce contracture, and allow the scar to fall into natural facial creases. This technique can improve both function and appearance as the child grows. [Z-plasty]

3. W-plasty or straight-line closure
Some surgeons prefer a W-plasty or carefully placed straight-line incision depending on the child’s facial creases and the extent of the cleft. The purpose is similar: a fine, stable scar that does not pull the corner of the mouth laterally. Long-term outcome studies compare these techniques but agree that muscle repair quality is more important than skin pattern alone. [Surgical Techniques]

4. Vermilion square or vermilion flap techniques
Special flaps taken from the red part of the lip (vermilion) help recreate a natural-looking vermilion at the new commissure. In vermilion square flap repair, a small square of vermilion is rotated to form the corner. The purpose is to avoid a “blunt” or pale mouth corner and instead create a sharp, natural lip border. [Vermilion Square Flap]

5. Revision and secondary contouring surgeries
As the child’s face grows, subtle asymmetry or scar tightness may appear. Minor revision surgery can fine-tune the commissure position, release contractures, or adjust lip thickness. The purpose is to maintain symmetry and function into adolescence and adulthood. Planning is individualized and usually done after growth spurts. [Long-term Outcomes]

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Prevention

Macrostomia itself is usually not preventable, because it arises early in facial development and often has no clear single cause. However, families can focus on preventing complications and supporting healthy pregnancy and childhood:

1. Healthy pre-pregnancy and pregnancy care – Folic acid, avoidance of smoking, alcohol, and illicit drugs, and good prenatal care support overall fetal development, though they cannot guarantee prevention of macrostomia. [Maternal Health]

2. Avoidance of known teratogens – Some medicines or environmental exposures can harm developing facial structures; obstetricians review medications to minimize risk. [Teratology]

3. Genetic counselling when there is a family history – If macrostomia occurs with a syndrome or runs in families, genetic specialists can discuss recurrence risk and options for future pregnancies. [Genetics]

4. Early diagnosis and referral to a craniofacial team – Prompt referral soon after birth prevents feeding problems and allows timely planning of surgery and therapies. [Craniofacial Care]

5. Preventing malnutrition in infancy – Early feeding support avoids growth failure, which could delay surgery and worsen outcomes. [Feeding Support]

6. Preventing wound infection after surgery – Following wound-care instructions and, where appropriate, using perioperative antibiotics lowers infection risk. [Antibiotic Prophylaxis]

7. Preventing scar contracture – Regular scar massage, sun protection, and follow-up help prevent tight, pulling scars at the mouth corner. [Scar Care]

8. Preventing dental disease – Good brushing, fluoride, and regular dental visits prevent tooth decay that can complicate prosthetic or orthodontic work. [Dental Care]

9. Preventing psychological harm – Early psychosocial support, anti-bullying measures, and age-appropriate explanations help prevent long-term emotional trauma. [Mental Health]

10. Preventing unsafe, unproven treatments – Families should avoid non-medical “stem-cell” or cosmetic procedures that lack scientific backing and may be harmful. [Regulatory Advice]

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When to see doctors

Parents or older children should contact their craniofacial team or pediatrician urgently if they notice:

• Feeding difficulty, choking, or poor weight gain, especially in infancy, as these can signal unsafe swallowing and risk of aspiration pneumonia. [Pediatric Feeding]

• Breathing problems, noisy breathing, or repeated chest infections, which may indicate aspiration or airway issues. [Respiratory Risk]

• Signs of wound infection after surgery such as increasing redness, swelling, pus, bad smell, or fever. [Postoperative Care]

• Sudden opening of the surgical wound or separation of stitches at the mouth corner, which may need prompt surgical review. [Surgical Complication]

• Increasing drooling, speech regression, or difficulty closing the mouth, which could suggest scar contracture or muscle imbalance. [Functional Follow-up]

• Severe pain not relieved by prescribed medicines, especially if associated with swelling or fever. [Pain Management]

• Bullying, sadness, or withdrawal related to appearance, so that psychological support can be offered early. [Psychosocial Support]

Routine follow-up with the craniofacial team is also important even when there are no urgent problems, because the face changes with growth and small issues are easier to correct early.

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What to eat and what to avoid

After macrostomia repair, diet should be soft, nutritious, and gentle on the incision until the surgeon allows normal chewing.

What to eat
Soft, high-protein foods such as yogurt, mashed beans, eggs, soft rice, well-cooked pasta, smoothies, and pureed fruits and vegetables are usually recommended. These foods are easier to swallow, less likely to damage stitches, and provide the protein, vitamins, and minerals needed for healing. Cool or room-temperature foods can be soothing. Dietitians emphasize regular small meals to maintain energy and growth. [Pediatric Wound Nutrition]

What to avoid
In the early postoperative period, children are usually advised to avoid very hard, crunchy, or sharp foods (chips, crusty bread, nuts), as well as very hot or spicy dishes that can irritate the wound. Sticky sweets and sugary drinks should be limited because they promote tooth decay and may adhere to stitches. Chewing on toys or fingers should also be discouraged to protect the repair. Specific timelines are given by the surgeon based on the operation. [Diet After Oral Surgery]

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Frequently asked questions (FAQs)

1. Is macrostomia life-threatening?
On its own, macrostomia is usually not life-threatening, but it can cause feeding, speech, dental, and psychosocial problems if left untreated. When part of a broader syndrome, other organ systems may be involved, so overall risk depends on associated conditions. Early assessment by a craniofacial team ensures that any breathing or feeding risks are managed safely. [Craniofacial Review]

2. What causes macrostomia?
The exact cause is often unknown. It is thought to result from incomplete fusion of facial processes during early fetal development. Sometimes it occurs with conditions such as hemifacial microsomia or other craniofacial syndromes, suggesting genetic and environmental influences. Most parents did nothing wrong to “cause” it, and recurrence risk is usually low but can be discussed with a genetic counsellor. [Embryology]

3. At what age is surgery usually done?
Many surgeons plan repair in late infancy or early childhood, balancing the benefits of early function and appearance with the safety of anesthesia and the child’s overall health. Some repair isolated macrostomia around 6–12 months of age, sometimes timed alongside other facial surgeries, but decisions are individualized. [Surgical Timing]

4. How successful is macrostomia surgery?
Published case series report generally good functional and aesthetic outcomes with modern techniques. Success means a symmetrical mouth corner, good lip seal, and discreet scars that blend with natural creases. Minor revisions may be needed as the child grows, but most children eat, speak, and smile normally after treatment. [Outcome Studies]

5. Will my child need more than one operation?
Often, a single well-planned operation is enough for basic repair. However, growth changes and individual healing mean some children benefit from small secondary procedures for scar refinement or symmetry adjustments. Regular follow-up helps the team decide if and when revision is helpful. [Long-Term Follow-up]

6. Does macrostomia always come with other anomalies?
No. Macrostomia can be isolated or part of a syndrome. Some series show it frequently associated with ear anomalies, mandibular hypoplasia, or other facial clefts, while other reports describe isolated cases. Detailed clinical and sometimes imaging assessment looks for associated differences that may influence treatment. [Case Series]

7. Will my child be able to speak normally?
Most children develop normal or near-normal speech with a combination of good surgical repair and, where needed, speech therapy. Early detection of articulation problems and consistent practice are important. Some sounds that depend heavily on precise lip closure may need extra attention in therapy. [Speech Outcomes]

8. Is breastfeeding possible in babies with macrostomia?
Breastfeeding can be more difficult because the baby may not form a tight seal around the nipple. Lactation consultants and feeding therapists may help adapt positions or recommend pumping and specialized bottles. The main goal is safe, adequate nutrition, whether from breast milk via bottle or formula. [Feeding Guidance]

9. Will there be a visible scar?
Yes, any surgery leaves a scar, but surgeons design incisions to lie along natural facial lines and use techniques such as Z-plasty or W-plasty to break up the line. With time, scar care, and sun protection, many scars fade and become much less noticeable. [Scar Outcomes]

10. Are there risks from the surgery?
As with any operation, risks include bleeding, infection, wound breakdown, asymmetric commissure, and unsatisfactory scars. Anesthesia also carries small risks, which are carefully managed by pediatric anesthesiologists. Thorough preoperative assessment and experienced surgical teams help keep complication rates low. [Risk Discussion]

11. Do children with macrostomia have normal intelligence?
Most children with isolated macrostomia have normal intelligence and development. If macrostomia is part of a broader syndrome, there may be associated developmental issues, but these relate to the underlying syndrome rather than the cleft itself. Early developmental screening ensures support if needed. [Development]

12. Can macrostomia come back after surgery?
The cleft itself does not “come back,” but scars can tighten or grow less favorably, causing mild pulling of the commissure. Growth of the face can also reveal subtle asymmetry later. These changes can often be improved with therapy or small revision procedures. [Revision Surgery]

13. Is there any role for orthodontics later?
Yes. As teeth erupt and jaws grow, orthodontic evaluation is important to correct bite problems, align teeth, and coordinate dental arches with the reconstructed lip and cheek. Orthodontic treatment is often part of the broader craniofacial plan in adolescence. [Orthodontic Care]

14. Are strong pain medicines always necessary after macrostomia repair?
Not always. Many studies in pediatric cleft and craniofacial surgery show that non-opioid regimens with acetaminophen and NSAIDs, plus local nerve blocks, can provide good pain control and reduce or eliminate the need for opioids. When opioids are used, they are given at the lowest effective dose and for the shortest possible time under close monitoring. [Non-opioid Analgesia]

15. Where can families find reliable information and support?
Families can seek information and support from hospital-based craniofacial centers, reputable medical websites, and parent support organizations for craniofacial differences. Research articles on [PubMed] and guidelines from pediatric surgery and anesthesia societies give clinicians and families evidence-based information. It is best to avoid social media sources that offer unproven treatments or unrealistic promises. [Information Resources]

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: February 25, 2025.