Oksana Jackson

The Children's Hospital of Philadelphia modification of the Furlow double-opposing Z-palatoplasty: 30-year experience and long-term speech outcomes.

Background: The purpose of this study was to evaluate the long-term outcomes following modified Furlow palatoplasty over a 30-year experience at a single institution. Methods: A retrospective review was performed of all nonsyndromic patients undergoing primary modified Furlow palatoplasty at The Children’s Hospital of Philadelphia between 1980 and 2011. Surgical success was evaluated by the rate of postoperative oronasal fistula, speech outcomes assessed at a minimum of age 5 years, and the need for secondary surgery for velopharyngeal dysfunction. Results were analyzed by surgeon, Veau cleft type, age at repair, and over time. Results: Eight hundred sixty-nine patients who underwent modified Furlow repair were included in this study. The mean age at palatoplasty was 1.1 years, and the mean length of follow-up was 6.7 years. Repairs were performed by 11 surgeons, with 44.3 percent performed by a single surgeon. The overall oronasal fistula rate was 5.2 percent, and this improved significantly over the 30-year period (p = 0.003). Speech results at age 5 or older were available for 559 patients. Of these 559 patients, 72.4 percent had a competent velopharyngeal mechanism, and 21.5 percent had a borderline mechanism consistent with socially acceptable speech. Secondary pharyngeal surgery was indicated in 8.1 percent of patients. Speech results were stable (p = 0.10), and rates of secondary surgery for velopharyngeal dysfunction declined significantly over the study period (p = 0.005). Conclusions: The authors’ 30-year experience with the modified Furlow palatoplasty shows stable speech results, decreasing rates of secondary surgery, and an acceptable rate of postoperative oronasal fistula. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Repair of the immature and mature craniofacial skeleton with a carbonated calcium phosphate cement: Assessment of biocompatibility, osteoconductivity, and remodeling capacity

Background: The apatite compounds used most commonly in craniofacial reconstruction are highly crystalline and biologically inert ceramics. Because their capacity to be replaced by native bone is limited, they have found little application in repair of the growing craniofacial skeleton. Carbonated calcium phosphate cements more closely resemble the mineral phase of bone, thereby offering enhanced bioresorption and osteoconductivity, but their fate in the immature and mature craniofacial skeleton has not been investigated. Methods: The authors hypothesized that the capacity for cell-mediated remodeling of carbonated calcium phosphate cements is based on (1) their crystallographic and compositional similarity to the mineral phase of bone and (2) the osteogenic capacity of the host. Four noncritical-sized calvarial defects were created in six 3-week-old and six 16-week-old Yorkshire pigs. The defects were repaired with autologous bone, sintered carbonated calcium phosphate cement disks with a higher crystal order, or carbonated calcium phosphate cement (Norian CRS; Synthes Maxillofacial, West Chester, Pa.). The fourth defect was left empty as a control. Specimens were harvested at 30 and 90 days postoperatively. Results: Empty defects healed with dense fibroconnective tissue in all groups. Autologous bone grafts underwent complete remodeling and replacement with woven bone at both time points. Sintered carbonated calcium phosphate disks demonstrated no bony ingrowth or remodeling. In immature animals, carbonated calcium phosphate cement implants were progressively replaced with woven bone through osteoclast-mediated resorption and osteoblast-mediated bone formation. Only minimal remodeling of the carbonated calcium phosphate cement implants was observed in skeletally mature animals. Conclusions: The results of these exper-iments suggest that the extent of remodeling of carbonated calcium phosphate cement is dependent on both the composition of the implant itself and the osteogenic capacity of the host and that carbonated calcium phosphate cement may be used successfully for inlay applications in the immature craniofacial skeleton.

A 35-year experience with syndromic cleft palate repair: operative outcomes and long-term speech function.

BackgroundAssociated comorbidities can put syndromic patients with cleft palate at risk for poor speech outcomes. Reported rates of velopharyngeal insufficiency (VPI) vary from 8% to 64%, and need for secondary VPI surgery from 23% to 64%, with few studies providing long-term follow-up. The purpose of this study was to describe our institutional long-term experience with syndromic patients undergoing cleft palatoplasty. MethodsA retrospective review was conducted of all patients with syndromic diagnoses undergoing primary Furlow palatoplasty from 1975 to 2011. Outcomes included postoperative oronasal fistula (ONF) and need for secondary VPI surgery. Speech scores for verbal patients 5 years or older were collected via the Pittsburgh scale for speech assessment. Aggregate scores categorized the velopharyngeal mechanism as competent, borderline, or incompetent. Outcomes were analyzed by patient and operative factors. ResultsOne hundred thirty-two patients were included with average age at repair of 20.7 months. Cleft type was 9% submucosal, 16% Veau class I, 50% class II, 12% class III, and 13% class IV. Forty-five syndromes were recorded, most commonly Stickler syndrome (n = 32) and 22q11.2 deletion syndrome [22q11.2DS (n = 19)]. Forty-four patients also had associated Pierre Robin sequence (PRS). The overall ONF rate was 4.5% and was highest in Veau class IV clefts (P = 0.048). Seventy-six patients were included in speech analysis, with an average age at last assessment of 10.4 years. Overall, 60.5% of patients had a competent velopharyngeal mechanism, 23.7% borderline, and 15.8% incompetent mechanism. Fifty percent of 22q11.2DS patients had borderline speech and none had competent speech, compared to 73.3% with Stickler syndrome (P = 0.01) and 71.4% of patients with associated PRS (P = 0.02). Secondary VPI surgery was performed in 11.4% of patients overall. Patients with PRS (13.6%) and with Stickler syndrome (15.6%) had secondary VPI surgery, compared to 31.6% of patients with 22q11.2DS (P = 0.01). ConclusionsThis study demonstrates low rates of postoperative ONF after modified Furlow palatoplasty in syndromic patients. Speech outcomes were comparable to nonsyndromic patients at our institution, but patients with 22q11.2DS consistently had borderline-incompetent speech and a 3-fold higher incidence of secondary VPI surgery.

Anatomical dissection and modified Sihler stain of the lower branches of the facial nerve.

Background: The cervical branch of the facial nerve and the muscles it supplies play a role in perioral function and neck aesthetics. Paralysis of the cervical branch in some patients may result in lip depressor deficiency and an asymmetric smile. It is unclear whether this is the result of platysmal weakness alone, or attributable to coinnervation of the lip depressor muscles by the cervical and marginal mandibular branches of the facial nerve. The purpose of this study was to provide a detailed description of the anatomy and function of the cervical branch to assist surgeons operating in the face and neck. Methods: Anatomical dissection of the marginal mandibular and cervical branches was performed in 20 hemifacial human cadaver specimens. The modified Sihler stain was used to stain the extramuscular and intramuscular nerve branches in eight specimens. The course, branching patterns, and neuromuscular relationships of the cervical and marginal mandibular branches were examined. Results: Multiple cervical branches were identified in 85 percent of specimens, and an anastomosis with the transverse cervical cutaneous nerve was documented consistently. A communication between the marginal mandibular and cervical branches was identified in seven specimens, and coinnervation of the depressor anguli oris by intramuscular cervical branch ramifications was seen in one. Conclusions: A detailed description of the anatomy of the cervical branch is provided. Coinnervation of the lower lip depressors by the cervical branch remains a plausible explanation for the lower lip deformity attributed to cervical branch paralysis in some patients.

Do patients with Pierre Robin sequence have worse outcomes after cleft palate surgery

BackgroundPierre Robin sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, airway obstruction, and often clefting of the soft and hard palate. The purpose of this study was to compare oronasal fistula rates, long-term speech outcomes, and rates of secondary surgery for velopharyngeal insufficiency (VPI) after primary cleft palate repair using the modified Furlow technique between nonsyndromic patients with and without PRS. This study also sought to determine if type of airway management in infancy correlated with speech outcomes in patients with PRS. MethodsA retrospective review was performed of all nonsyndromic patients with clefts of the palate only who underwent repair between 1981 and 2006 at The Children’s Hospital of Philadelphia using the modified Furlow technique. Patient outcomes were evaluated by the rate of postoperative oronasal fistula, speech scores at a minimum of age 5 years using the Pittsburgh Weighted Values for Speech Symptoms Associated with VPI, and the need for secondary pharyngeal surgery for VPI. ResultsFifty-five patients with PRS and 129 without PRS were included in this study. There was no significant difference in oronasal fistula rate between groups. Speech outcomes were worse in patients with PRS with 52.7% demonstrating a competent velopharyngeal mechanism, and 30.9% a borderline mechanism, compared to 72.1% and 19.4% in the non-PRS group, respectively (P = 0.035). In addition, only 76.3% of patients with PRS had no or mild hypernasality compared to 91.5% of patients without PRS (P = 0.01). The rates of nasal emission, articulation errors associated with VPI, and secondary surgery for VPI did not differ between groups. Of the patients with PRS, 36 were managed in infancy with positioning alone and 15 underwent surgical intervention for airway obstruction, and there were no significant differences in speech scores or rates of secondary surgery for VPI between these subgroups. ConclusionsNonsyndromic patients with PRS had worse speech outcomes after modified Furlow cleft palate repair, but no significant differences were seen in the rates of secondary surgery for VPI or postoperative oronasal fistula. In addition, initial airway management in patients with PRS did not correlate with speech outcomes.

Perioperative risk factors for adverse airway events in patients undergoing cleft palate repair.

Objective To establish the incidence of perioperative airway complications in a large series of pediatric patients undergoing palatoplasty and to identify which specific patient, procedural, and provider factors are associated with increased risk for perioperative adverse airway events (AAEs). Design Retrospective chart review. Setting Tertiary pediatric hospital. Patients Included were 300 patients who underwent primary cleft palate repair using the modified Furlow technique between 2008 and 2011. Patients were 2 years or younger at the time of the operation. Main Outcome Measure(s) Charts were reviewed for perioperative AAEs, which were defined as postoperative airway obstruction, oxyhemoglobin saturation ≤85% for ≥45 seconds, bronchospasm, laryngospasm, reintubation, and unplanned admission to the intensive care unit. Patient-specific factors (diagnosis of a craniofacial syndrome, Veau cleft type, preoperative pulmonary and airway history), procedural factors (operative time, anesthesia time, opioid dose, administration and reversal of neuromuscular blockers), and provider factors (experience, number of providers), were documented, and associations with AAEs were investigated. Results AAEs occurred in 23% of patients overall and were significantly more common in syndromic patients (P = .003), patients with jaw or tracheal anomalies (P = .001), and patients with a history of difficult airway (P = .001). Other significant factors included prior history of difficult intubation (P = .05), surgeon (P = .02) and anesthesiologist experience (P = .05), and operative time (P = .02). Conclusions Diagnosis of a craniofacial syndrome, a history of preoperative airway problems, and provider inexperience correlated with increased risk for airway complications after palatoplasty. Recognizing patients at risk for AAEs may permit improved preoperative planning to optimize surgical outcomes and minimize complications.

Asymmetric Crying Facies in the 22q11.2 Deletion Syndrome Implications for Future Screening

Objective. Asymmetric crying facies (ACF) is congenital hypoplasia of the depressor anguli oris muscle characterized by asymmetry of lower lip depression during crying. This has an overall incidence of 0.6%. This study determines the incidence of ACF in a large population of patients with 22q11.2 deletion. Patients and Methods. A retrospective review of medical records on patients with a confirmed 22q11.2 deletion was undertaken. Results. A total of 836 records were reviewed. Of these, 117 (14%) were noted to have ACF on physical examination. Within this latter group, palatal anomalies were common (77%), as was congenital heart disease (78%); however, these numbers did not differ significantly from their known prevalence in the 22q11.2 population. Conclusions. We report a 14% incidence of ACF in patients with a 22q11.2 deletion, significantly higher than in the general population. We suggest, therefore, that newborns with ACF be referred for further screening for the 22q11.2 deletion syndrome.

Conjoined twin separation: lessons learned.

Background: The separation of conjoined twins presents a unique challenge to many pediatric surgical specialties. A multidisciplinary approach is essential for successful twin separation, and plastic surgeons are critical members of this surgical team when soft-tissue requirements preclude simple closure. Methods: In this article, the authors review multiple techniques available to plastic surgeons for providing adequate soft-tissue coverage in this unique clinical situation and present lessons learned from their experience at The Childrens Hospital of Philadelphia with seven conjoined twin separations performed between the years 1980 and 2007. Results: Successful separation was accomplished in six of the seven conjoined twin pairs, expander complications requiring reoperation occurred in three twin pairs, and skin grafting was required because of insufficient expansion in two twin pairs. The techniques found to be critical to successful separation include maximal extracavitary tissue expansion with smooth-wall remote port tissue expanders, protection of expanded skin through pressure-reducing strategies, fluorescein vascular territory mapping, the use of frozen banked autogenous skin for salvage, and the use of supplemental nutritional support. Conclusions: The overall success of conjoined twin separation depends on the experience and preparedness of the treating team and the resources available at the pediatric specialty center. Multiple techniques are available to plastic surgeons to optimize success in this challenging clinical situation. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

22q11.2 Deletion syndrome and obstructive sleep apnea

UNLABELLED Otolaryngologic problems are common in the 22q11.2 deletion syndrome (DS) population. Structural anomalies and retrognathia may predispose these patients to obstructive sleep apnea (OSA). The current association of OSA in this population is not defined. OBJECTIVE (1) Define the frequency of OSA in 22q11.2 DS patients referred for polysomnography (PSG). (2) Determine if OSA is present before and/or after surgery to correct velopharyngeal insufficiency (VPI). (3) Determine effect of prior adenotonsillectomy on OSA following VPI surgery. METHODS Retrospective review of children treated from 2006 to 2013 in a tertiary care setting identified by ICD-9 758.32 (velocardiofacial syndrome) and 279.11 (DiGeorge syndrome). Surgical history and PSG data were abstracted from the identified records. RESULTS We identified 323 patients with 22q11.2 DS; 57 (18%) were screened at any point in care using PSG and 15 patients had PSG at multiple time points in care. In most cases, indication for PSG was sleep disordered breathing or pre-operative planning. Overall, 33 patients met criteria for OSA on PSG, accounting for 10.2% of our study population; however, the percentage of patients with OSA was significantly higher within the group of 57 patients (58%) who were screened with PSG. Twenty-one of the screened patients (54%) had PSG prior to any pharyngeal surgery and had mild to severe OSA (obstructive apnea/hypopnea index (AHI): median 5.1/h, range 1.9-25.6). Eighteen patients had PSG after adenotonsillectomy; 8 of these patients (44%) had mild to moderate OSA (median AHI 2.95/h, range 1.9-5.4). Seventeen patients had PSG after VPI surgery (palatopharyngeal flap (PPF) n=16, sphincteroplasty n=1). Nine of these patients (53%) had mild to severe OSA (median AHI 3/h, range 1.9-15). Patients who underwent adenotonsillectomy prior to VPI surgery had similar prevalence of OSA (50%, n=12) than those who did not (OSA: 60%, n=5, p=0.70). Most children had mild OSA. CONCLUSION Prevalence of OSA in this population of 22q11.2 DS patients is higher than expected in the general population. OSA risk is highest after VPI surgery, and may be decreased by adenotonsillectomy. Providers should have awareness of increased prevalence of OSA in patients with 22q11.2 DS. Close monitoring for OSA is warranted given the likelihood of subsequent surgical intervention that can worsen OSA.

Perioperative Risk Factors in Patients With 22q11.2 Deletion Syndrome Requiring Surgery for Velopharyngeal Dysfunction

Objective To determine the prevalence of cardiac, cervical spine, and carotid artery abnormalities in patients with 22q11.2 deletion syndrome (22q11.2DS) undergoing surgery for velopharyngeal dysfunction (VPD), associations between the presence of these abnormalities, and whether these abnormalities caused changes in surgical management or perioperative complications. Design Retrospective review. Setting Tertiary pediatric hospital. Patients Seventy patients with 22q11.2DS with complete preoperative cervical vascular and spine imaging and cardiac evaluation between 1998 and 2011. Main Outcome Measures Incidence of cardiac, cervical spine, and vascular abnormalities; related perioperative complications; and resulting changes in surgical, anesthetic, or perioperative management plan. Results Cardiac abnormalities occurred in 45 patients (64.3%), and 8 patients required cardiac anesthesia. Thirty-eight patients (54.3%) had at least one vascular abnormality of the neck, and 14% had medial deviation of the internal carotid artery. Surgery was not performed in one patient, and the surgical plan was altered in three patients because of carotid anomalies. Cervical spine abnormalities were found in 24 patients (34.3%); 8 patients demonstrated radiographic evidence of cervical instability and were treated with spinal precautions during surgery. The presence of one anomaly was not predictive of any other finding, and there were no complications related to the heart, cervical spine, or carotid arteries. Conclusions Anomalies of the heart, cervical spine, and cervical vasculature occur frequently in 22q11.2DS, vary drastically in severity, and are impossible to predict based on other features of the syndrome. Preoperative diagnosis of these comorbidities with routine imaging can minimize the risk of avoidable surgical complications.